Chapter 38 - Neurosurgery

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Chapter 38: Neurosurgery
DIAGNOSIS & MANAGEMENT OF ALTERATIONS IN CONSCIOUSNESS

Kyle Wu, MD, & Timothy R. Smith, MD, PhD
Consciousness, the complex interplay between wakefulness and awareness, exists on a spectrum from “normal” consciousness to coma, and various
states in between. Acute confusional state (delirium) and encephalopathy are among the most common disorders of consciousness encountered in
acutely ill patients, especially those recovering from surgery or anesthetic medications or adapting to the hospital environment during the
postoperative period. The prevalence of altered mental status in hospitalized patients is high, with reported rates up to 55%, and is associated with
increased rates of mortality ranging from 10%-70%. Given the high incidence of altered mental states and their similarity at presentation to various
states of primary neurologic disease, an understanding of the pathophysiology, diagnosis, and management of common etiologies is warranted for all
medical practitioners.
DEFINITIONS
Consciousness is generally defined as a combination between the experience of the environment and self. It is composed of two components: arousal,
a measure of wakefulness, and awareness, a state of perception. This distinction is useful, since the two processes are dissociable. For example, a
vegetative state is characterized by a patient who is awake but not necessarily aware of external stimuli or his or her environment (and thus not
interactive). On the other hand, the unfortunate patient suffering from locked-in syndrome (due to a structural lesion of the brainstem) is both awake
and aware; however, he or she is incapable of movement.
Arousal is generated by activity of the ascending reticular activating system, which is composed of neurons within the central mesencephalic
brainstem, the lateral hypothalamus, and portions of the thalamus. Widespread projections of these nuclei synapse with neurons in the cerebral
cortex to generate an arousal response. The arousal response defines the level of consciousness (eg, being awake vs asleep vs comatose). Awareness
is thought to be generated through networks involving the thalamus and association cortices of the frontal, parietal, temporal, and occipital lobes.
Processes related to awareness define the content of consciousness (eg, seeing a blue circle vs a red triangle). It can be thought of as the way in which
one experiences his or her own state of being and relates this to incoming sensory stimuli.
Many terms are used to describe levels of consciousness ranging from alert to comatose. Here we will describe some of the more commonly used
terminology. An alert patient is awake and immediately responsive to all stimuli. Stupor is a condition in which the patient is less alert but still responds
appropriately to stimulation. An obtunded patient appears to be asleep much of the time but responds, albeit less briskly than expected, to noxious
stimuli. A vegetative state is a state of arousal without awareness in which the patient may open his or her eyes, track objects, chew, and swallow, but
not respond to auditory stimuli or appear to sense pain (although pain processing is known to occur in the vegetative state). The comatose patient
appears to be asleep and does not respond to stimuli. Often, terms used to describe states of consciousness lack consistent definitions, and a clear
description of a patient’s state of arousal and response to specific stimuli results in more precise communication.
PATHOPHYSIOLOGY OF ALTERED CONSCIOUSNESS
In general, altered states of consciousness occur due to insults to the key anatomic structures or pathways previously mentioned. Owing to the
bilateral organization of the brain, typically both cerebral hemispheres or thalami, or the reticular activating system within the brainstem itself, must
suffer injury to lead to altered states of consciousness. This can arise from causes that may be structural (eg, stroke, hemorrhage, trauma, infection,
seizure, tumor) or nonstructural (eg, pharmacologic or altered physiologic states). Structural disease, such as tumor or stroke, will often manifest with
a constellation of neurologic symptoms that relate to their anatomic location (alterations in speech, coordination, or movement). Nonstructural
disease, such as hypoglycemia, hypoxia, hypercarbia, hyponatremia, and hypothermia, is more likely to manifest with a globally depressed level of
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consciousness without a clear neurologic motor or sensory localization. Pharmacologic etiologies, such as intoxication or withdrawal, medication
Chapter 38: Neurosurgery, Timothy R. Smith Page 1 / 93
overdose, or residual anesthesia after surgery, should be considered and reversed when possible.
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Structural Pathology
In general, altered states of consciousness occur due to insults to the key anatomic structures or pathways previously mentioned. Owing to the
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bilateral organization of the brain, typically both cerebral hemispheres or thalami, or the reticular activating system within the brainstem itself, must
suffer injury to lead to altered states of consciousness. This can arise from causes that may be structural (eg, stroke, hemorrhage, trauma, infection,
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seizure, tumor) or nonstructural (eg, pharmacologic or altered physiologic states). Structural disease, such as tumor or stroke, will often manifest with
a constellation of neurologic symptoms that relate to their anatomic location (alterations in speech, coordination, or movement). Nonstructural
disease, such as hypoglycemia, hypoxia, hypercarbia, hyponatremia, and hypothermia, is more likely to manifest with a globally depressed level of
consciousness without a clear neurologic motor or sensory localization. Pharmacologic etiologies, such as intoxication or withdrawal, medication
overdose, or residual anesthesia after surgery, should be considered and reversed when possible.
Structural Pathology
Structural lesions cause altered consciousness through either insult to the bilateral cerebral hemispheres or thalami or the reticular activating region
of the brainstem. Given the anatomic density of brainstem nuclei (ie, a cluster of neurons with similar functions and shared connections), structural
lesions affecting the brainstem typically impact multiple functions and manifest with multiple neurologic deficits including cranial neuropathy.
Common structural lesions include stroke (both hemorrhagic or ischemic), intracranial hematoma (epidural, subdural, or intraparenchymal),
infections (eg, cerebral abscess), or neoplasms.
The reticular activating system is excited by a wide variety of stimuli, particularly somatosensory stimuli. Given that its nuclei are highly concentrated in
the midbrain, it may be damaged by central midbrain lesions, resulting in decrease or loss of arousal. Less severe dysfunction of the reticular activating
system results in acute confusional state. The cardinal clinical signs of acute confusional state are somnolence, inattention, and disorientation,
sometimes with the addition of insomnia and agitation. In addition, perceptions may be distorted, leading to hallucinations and inability to organize
and interpret complex stimuli. Disordered perception results in dysfunctional learning, memory, and problem solving. Thought processes can be
disorganized and tangential, and the confused patient may develop delusions. In some cases, the acute confusional state presents as delirium, which
can, in its hyperactive form, be characterized by heightened arousal, disordered perception, agitation, aggression, delusions, hallucinations, and
autonomic hyperactivity (diaphoresis, tachycardia, hypertension, mydriasis). However, it is important to note that hypoactive delirium is the more
common variant to manifest in the postoperative setting.
Neurons in the hypothalamus, basal ganglia, midbrain, and pons are thought to be the primary anatomic structures involved in regulation of sleep-
wake cycles, as opposed to the midbrain’s regulation of arousal. Therefore, lesions involving the pons may preserve a patient’s ability to arouse to
stimuli but disturb sleep-wake cycles. This contrasts with the typical vegetative state that results from diffuse destruction or injury of the bilateral
cerebral cortices (often secondary to global cerebral ischemia) with preservation of the brainstem reticular activating system and sleep centers,
leading to a situation of near-normal wakefulness but complete loss of awareness. This leaves the patient with preserved sleep-wake cycles without
the ability to interact with the environment.
Nonstructural Pathology
Several nonstructural disorders that diffusely disturb brain function can produce a confusional state or, if severe, may result in coma (Table 38–1).
Acute toxic-metabolic encephalopathy (TME), which encompasses delirium and the acute confusional state, is an acute condition of global cerebral
dysfunction in the absence of primary structural brain disease. Typically, TME is a reversible consequence of systemic disease, infection, drugs, or
metabolic toxins, making prompt recognition and treatment critical.
Table 38–1.
Etiology of altered state of consciousness.
Structural Nonstructural
Trauma: Diffuse axonal injury, contusion, subdural or epidural hematoma Medication or pharmacologic overdose
Neoplasm (metastatic, primary, or leptomeningeal involvement) Medication or pharmacologic withdrawal
Infection: meningitis or encephalitis Alcohol intoxication
Vascular Hepatic encephalopathy
Ischemic stroke Infection
Hemorrhagic stroke (intraparenchymal hemorrhage) Systemic inflammatory response syndrome/sepsis
Ruptured aneurysm, cavernous malformation, or arteriovenous malformation Global cerebral ischemia
Seizures Myocardial Infarction
Subclinical status epilepticus Pulmonary embolus
Prolonged postictal state (Todd paresis) Insomnia/iatrogenic sleep prevention
Hydrocephalus Hypoxia/hypercarbia
Chapter 38: Neurosurgery, Timothy R. Smith
Communicating Hyperosmolar state Page 2 / 93
Noncommunicating Hypo-/hypercalcemia
Hypo-/hyperthermia
Several nonstructural disorders that diffusely disturb brain function can produce a confusional state or, if severe, may result in coma (Table 38–1).
Acute toxic-metabolic encephalopathy (TME), which encompasses delirium and the acute confusional state, is an acute condition of global cerebral
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dysfunction in the absence of primary structural brain disease. Typically, TME is a reversible consequence of systemic disease, infection, drugs, or
metabolic toxins, making prompt recognition and treatment critical.
Table 38–1.
Etiology of altered state of consciousness.
Structural Nonstructural
Trauma: Diffuse axonal injury, contusion, subdural or epidural hematoma Medication or pharmacologic overdose
Neoplasm (metastatic, primary, or leptomeningeal involvement) Medication or pharmacologic withdrawal
Infection: meningitis or encephalitis Alcohol intoxication
Vascular Hepatic encephalopathy
Ischemic stroke Infection
Hemorrhagic stroke (intraparenchymal hemorrhage) Systemic inflammatory response syndrome/sepsis
Ruptured aneurysm, cavernous malformation, or arteriovenous malformation Global cerebral ischemia
Seizures Myocardial Infarction
Subclinical status epilepticus Pulmonary embolus
Prolonged postictal state (Todd paresis) Insomnia/iatrogenic sleep prevention
Hydrocephalus Hypoxia/hypercarbia
Communicating Hyperosmolar state
Noncommunicating Hypo-/hypercalcemia
Hypo-/hyperthermia
Hypo-/hyperglycemia
Hypo-/hypernatremia
Hypo-/hyperthyroidism
Uremia
TME is common among critically ill patients and likely underdiagnosed, especially when occurring in patients who require mechanical ventilation. Risk
factors for the development of TME include admission to an intensive care unit (ICU), prolonged hospitalization, advanced age, preexisting primary
neurologic disorders, nutritional deficiency, infection, temperature dysregulation, and failure of multiple organ systems.
PATHOPHYSIOLOGY OF ELEVATED INTRACRANIAL PRESSURE
The Monro-Kellie doctrine states that the cranium contains three major components: brain parenchyma, cerebrospinal fluid (CSF), and blood. When
healthy, the volumetric sum of these components is maintained at a constant within the rigid and nonexpansile cranial vault. However, in states of
pathology, the doctrine states that an increase in one component must be offset by a decrease in another, or else intracranial pressure (ICP) will
increase. This offset is normally accomplished by displacement of CSF into the contiguous subarachnoid space of the spinal cord, termed spatial
compensation. This delicate balance, however, has physiologic limits. Once these compensatory limits are met, an increase in the volume of any
component will lead to an exponential increase in ICP, as illustrated by the intracranial elastance curve (Figure 38–1). If pressure in the intracranial
compartment increases to nonphysiologic levels, the brain is displaced out of its typical compartment, resulting in “herniation” into another
compartment.
Figure 38–1.
Intracranial compliance curve demonstrating the approximate relationship between intracranial pressure (ICP) and an expanding intracranial mass.
compartment.
Figure 38–1.
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Intracranial compliance curve demonstrating the approximate relationship between intracranial pressure (ICP) and an expanding intracranial mass.
Normal ICP in adults is typically cited to be < 10-15 mm Hg. Disease states such as the presence of tumor or blood, the impedance of CSF drainage
(hydrocephalus), cerebral edema (commonly due to tumor or stroke), or increased cerebral blood flow (hyperemia as a response to head injury or
hypoventilation causing hypercarbia and vasodilation) may raise the ICP to a dangerous level. This creates a positive feedback cycle where increased
ICP leads to reduced cerebral perfusion and cytotoxic edema due to neuronal anoxic injury, resulting in worsening edema and further increase in ICP.
Furthermore, mass effect from swelling may progress to shifting of the brain parenchyma and devastating herniation.
SIGNS & SYMPTOMS OF INCREASED ICP
Signs and symptoms of increased ICP include altered mental status, headache, vomiting, and papilledema on fundoscopic examination. Cushing triad
may be seen in patients with severely increased ICP and consists of hypertension, bradycardia, and respiratory irregularity. Obtundation or focal
neurologic findings including unilateral pupillary dilation from pressure or traction exerted on the third cranial nerve (ie, the “blown” pupil) are late
findings indicative of impending uncal herniation.
Cerebral Herniation Syndromes
Cerebral herniation occurs when increased ICP results in a portion of the brain shifting from one intracranial compartment to another. These shifts
may lead to vascular compromise and infarction of portions of the brain or brainstem, disruption of white matter pathways carrying motor and
sensory signals, or direct pressure on structures such as cranial nerve III.
Subfalcine Herniation
Subfalcine herniation occurs in patients with a frontal lobe mass causing the cingulate gyrus to herniate beneath the falx into the contralateral frontal
lobe. Occasionally, this may result in anterior cerebral artery compression and subsequently ischemic stroke (manifesting as leg weakness).
Uncal Herniation
Uncal herniation occurs in patients with an expanding supratentorial mass. Early symptoms include contralateral hemiparesis, diminished
consciousness, and an ipsilateral cranial nerve III palsy. Compression of the Edinger-Westphal nucleus leads to dilation of the pupil due to loss of
parasympathetic input. As further herniation and midbrain/reticular activating system compression occur, somnolence ensues. Late-stage herniation
may eventually result in brain death and eventual cardiopulmonary collapse.
Cerebellar Tonsillar Herniation
Posterior fossa masses cause symptoms by compressing the brainstem and obstructing the outward flow of CSF (hydrocephalus). As ICP increases in
the posterior fossa, the cerebellar tonsils may be pushed into or through the foramen magnum. As the medulla is compressed, apnea results from
dysfunction of the medullary respiratory center, ultimately leading to cardiopulmonary arrest.
Upward Transtentorial Herniation
Posterior fossa masses may cause obstructive hydrocephalus, leading to increased pressure in the posterior fossa (ie, infratentorial compartment)
that is greater than the pressure of the supratentorial compartment, which in turn leads to upward herniation of the posterior fossa contents,
including cerebellum and brainstem. This leads to brainstem dysfunction and eventually brain death.
DIAGNOSTIC EVALUATION OF ALTERED CONSCIOUSNESS
The diagnosis of an altered state of consciousness is provided by general physical examination, neuroradiologic imaging, drug screens, and laboratory
dysfunction of the medullary respiratory center, ultimately leading to cardiopulmonary arrest.
Upward Transtentorial Herniation
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Posterior fossa masses may cause obstructive hydrocephalus, leading to increased pressure in the posterior fossa (ie, infratentorial compartment)
that is greater than the pressure of the supratentorial compartment, which in turn leads to upward herniation of the posterior fossa contents,
including cerebellum and brainstem. This leads to brainstem dysfunction and eventually brain death.
DIAGNOSTIC EVALUATION OF ALTERED CONSCIOUSNESS
The diagnosis of an altered state of consciousness is provided by general physical examination, neuroradiologic imaging, drug screens, and laboratory
studies.
Neurologic Examination
The neurologic exam includes assessment of level of consciousness, brainstem reflexes, and motor activity. Rapid, standardized assessment of the
level of consciousness is useful for clinical decision making and communication. The level of alertness reflects the severity of the underlying condition;
severely affected patients are comatose. Commonly, the Glasgow Coma Scale (GCS) (Table 38–2) is used to quickly assess and communicate the level of
consciousness of a patient. It was initially developed for patients suffering from head trauma, but it is also widely used as assessment of level
consciousness regardless of etiology. A patient with a GCS score ≤ 8 is generally considered severe, possibly requiring intubation for ventilation. As
opposed to obtundation or somnolence, the cardinal feature of acute confusional state is impaired attention. Simple bedside tasks such as serial
subtraction or naming the months of the year in reverse can be used to test attention. Marked fluctuations in mental status over time are characteristic.
Other common findings include a disturbed sleep-wake cycle, decreased alertness, hypervigilance, hallucinations, sensory misperceptions, impaired
memory, and disorientation. The thought process is often disorganized, manifested by confused or rambling conversation.
Table 38–2.
Glasgow Coma Scale.
Eye opening:
Spontaneous 4
To voice 3
To pain 2
None 1
Verbal response:
Oriented 5
Confused, disoriented 4
Inappropriate words 3
Incomprehensible sounds 2
None 1
Best motor response:
Obeys 6
Localizes 5
Withdraws (flexion) 4
Abnormal flexion posturing

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Extension posturing 2
None 1
opposed to obtundation or somnolence, the cardinal feature of acute confusional state is impaired attention. Simple bedside tasks such as serial
subtraction or naming the months of the year in reverse can be used to test attention. Marked fluctuations in mental status over time are characteristic.
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Other common findings include a disturbed sleep-wake cycle, decreased alertness, hypervigilance, hallucinations, sensory misperceptions, impaired
memory, and disorientation. The thought process is often disorganized, manifested by confused or rambling conversation.
Table 38–2.
Glasgow Coma Scale.
Eye opening:
Spontaneous 4
To voice 3
To pain 2
None 1
Verbal response:
Oriented 5
Confused, disoriented 4
Inappropriate words 3
Incomprehensible sounds 2
None 1
Best motor response:
Obeys 6
Localizes 5
Withdraws (flexion) 4
Abnormal flexion posturing 3
Extension posturing 2
None 1
The cranial nerves and their corresponding brainstem reflexes, including pupillary response to light, corneal reflexes, oculocephalic reflex (doll’s
eyes), vestibulo-ocular reflex (cold calorics), breathing patterns, and the cough and gag reflexes, are used to describe accurately the neurologic status
and localize the level of brainstem dysfunction. Generally, brainstem reflexes are only affected in severe TME or with structural brainstem lesions.
Abnormal patterns of respiration, particularly Cheyne-Stokes respiration, may also occur with significant brainstem dysfunction.
Motor responses may be categorized as either spontaneous or induced by noxious stimuli, purposeful or nonpurposeful, unilateral or bilateral, or
involving either the upper or lower extremities. The patient may display withdrawal from a stimulus, abnormal flexion (decorticate posturing),
abnormal extension (decerebrate posturing), or absence of motor activity. Both types of posturing are indicative of corticospinal tract damage. A
variety of motor abnormalities are also associated with underlying TME; tremor, asterixis, multifocal myoclonus (sudden, nonrhythmic, gross muscle
twitching), paratonia (increased tone with variable resistance), diffuse brisk deep tendon reflexes, or bilateral extensor plantar responses may be
indicative of a particular underlying pathology.
Laboratory Studies
The laboratory workup for decreased level of consciousness is focused mainly on sorting out toxic-metabolic abnormalities. Basic studies include a
complete blood count, coagulation studies, complete metabolic panel, calcium, magnesium, phosphate, glucose, blood urea nitrogen, creatinine,
involving either the upper or lower extremities. The patient may display withdrawal from a stimulus, abnormal flexion (decorticate posturing),
abnormal extension (decerebrate posturing), or absence of motor activity. Both types of posturing are indicative of corticospinal tract damage. A
variety of motor abnormalities are also associated with underlying TME; tremor, asterixis, multifocal myoclonus (sudden, nonrhythmic, gross muscle
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twitching), paratonia (increased tone with variable resistance), diffuse brisk deep tendon reflexes, or bilateral extensor plantar responses may be
indicative of a particular underlying pathology.
Laboratory Studies
The laboratory workup for decreased level of consciousness is focused mainly on sorting out toxic-metabolic abnormalities. Basic studies include a
complete blood count, coagulation studies, complete metabolic panel, calcium, magnesium, phosphate, glucose, blood urea nitrogen, creatinine,
bilirubin, liver enzymes, ammonia, serum osmolality, and arterial blood gases. Toxicology screening should be performed for suspected intoxications.
Blood, sputum, urine, or CSF cultures should be considered if there is concern for infection. Further analysis of CSF with white and red blood cell
counts, protein, and glucose may suggest an infectious or neoplastic process. Targeted studies may also be performed such as those for encephalitis
or meningitis (eg, herpes simplex virus polymerase chain reaction, fungal cultures) or neoplastic processes (eg, cytology, flow cytometry). Thyroid
function tests and vitamin B12 and serum cortisol concentrations should be assessed if endocrinopathy is suspected.
Neuroradiologic Studies
Emergent computed tomography (CT) imaging to assess for structural lesions (eg, intracerebral hemorrhage, large territory ischemic stroke,
hydrocephalus, neoplasm, or diffuse cerebral edema) is strongly recommended for patients presenting with focal neurologic deficits or severe decline
in level of consciousness. Acute ischemic stroke within the first 3-4 hours may be clinically occult on CT imaging. Magnetic resonance imaging (MRI) of
the head is generally reserved to further assess for acute ischemic stroke as well as further characterization of neoplastic lesions. MRI should not be
relied upon for an emergent diagnosis given the time required to obtain the study.
Electroencephalography
The electroencephalogram (EEG) can both confirm global cerebral dysfunction and exclude subclinical status epilepticus with greater sensitivity than
clinical examination alone. EEG should be considered in patients with altered consciousness without a clear structural or toxic-metabolic etiology. The
degree of diffuse slowing of the normal background plus abnormal mixed rhythms in the EEG correlates with the severity of TME. Slowing can be
categorized as follows: mild, with a reduction in the normal alpha frequencies (8-13 Hz); moderate, with theta frequencies (4-8 Hz); and severe, delta
frequencies (< 4 Hz).
ICP Monitoring
Quantification of ICP is critical for management of patients with large intracerebral lesions resulting in mass effect and shift of midline structures,
diffuse cerebral edema, or hydrocephalus. ICP cannot accurately be estimated based on clinical findings or imaging. There are several methods of
direct ICP measurement, but the two most commonly employed in clinical practice are ventricular catheters and intraparenchymal microtransducer
“bolt monitor” systems. Other methods such as subarachnoid and epidural devices have much lower accuracy.
The gold standard of ICP monitoring is via an intraventricular catheter connected to a standard pressure transducer. These catheters are usually
placed into the lateral ventricle via a small frontal burr hole. The advantages of intraventricular catheters are that they measure global ICP, allow for
therapeutic drainage of CSF to lower ICP, and are amenable to external calibration. Disadvantages include risk for infection, catheter-induced
hematoma, or misplacement after insertion due to the blind nature of the procedure.
Microtransducer-tipped ICP monitors are most commonly placed in the brain parenchyma through a skull bolt. They are almost as accurate as
intraventricular catheters and have the advantages of lower infection and complication rates. The major disadvantages are an inability to drain CSF
and inability to recalibrate after placement, with a slight drift of measurements over time. They are generally reserved for situations of trauma or
altered consciousness to guide further treatments. Because they are purely diagnostic, in cases where increased ICP is suspected, external ventricular
catheter placement is favored.
MANAGEMENT OF ALTERED CONSCIOUSNESS
The initial treatment of a patient with an alteration in consciousness must employ management of the ABCs: airway, breathing, and circulation.
Patients who are not responsive enough to protect their own airway require intubation to reduce the risk of aspiration of gastric contents. During
intubation, hypoxia and hypotension should be avoided in brain-injured patients and a high suspicion for cervical spine injury should be maintained in
cases of trauma. A patient presenting with a GCS score ≤ 8 may require intubation for airway protection and to provide adequate oxygenation and
ventilation. Patients presenting with hypotension and shock must be aggressively treated with fluids and vasopressors to maintain adequate
perfusion. Sources of shock (septic, cardiogenic, or hypovolemic) should be investigated and treated appropriately.
Regardless of the cause of acute decline in mental status, a number of general measures should be instituted. There should be a discontinuation of all
drugs with potential toxicity to the CNS, if possible. Antipsychotic medications such as haloperidol or quetiapine can be used for management of
severe agitation. Thiamine should be administered to patients with a history of alcoholism, malnutrition, cancer, hyperemesis gravidarum, or renal
The initial treatment of a patient with an alteration in consciousness must employ management of the ABCs: airway, breathing, and circulation.
Patients who are not responsive enough to protect their own airway require intubation to reduce the risk of aspiration of gastric contents. During
intubation, hypoxia and hypotension should be avoided in brain-injured patients and a high suspicion for cervical spineAccess Provided by:
injury should be maintained in
cases of trauma. A patient presenting with a GCS score ≤ 8 may require intubation for airway protection and to provide adequate oxygenation and
ventilation. Patients presenting with hypotension and shock must be aggressively treated with fluids and vasopressors to maintain adequate
perfusion. Sources of shock (septic, cardiogenic, or hypovolemic) should be investigated and treated appropriately.
Regardless of the cause of acute decline in mental status, a number of general measures should be instituted. There should be a discontinuation of all
drugs with potential toxicity to the CNS, if possible. Antipsychotic medications such as haloperidol or quetiapine can be used for management of
severe agitation. Thiamine should be administered to patients with a history of alcoholism, malnutrition, cancer, hyperemesis gravidarum, or renal
failure on hemodialysis in order to prevent the development of Wernicke encephalopathy.
In the event that a patient presents with history or examination findings suggestive of elevated ICP, immediate life-saving measures may be required
prior to a detailed laboratory workup. These situations generally rely upon clinical judgment, and expert consultation with a neurologist or
neurosurgeon is strongly recommended. An examination consistent with a critically elevated ICP (coma with GCS score < 8, unilateral or bilaterally
fixed and dilated pupil[s], decorticate or decerebrate posturing, or Cushing triad of bradycardia, hypertension, and respiratory depression) warrants
immediate intervention while delaying additional diagnostic studies. In addition to standard resuscitation measures, other measures include elevation
of the head above the heart (usually 30 degrees) to increase cerebral venous outflow, temporary hyperventilation to a goal PaCO2 of 26 to 30 mm Hg,
and administration of intravenous hyperosmolar therapy (either mannitol or hypertonic saline infusion). Immediately following these measures, rapid
evaluation of the underlying diagnosis by obtaining a thorough history, detailed neurologic examination, and neuroradiologic imaging should be
pursued. Neurosurgical consultation should be considered for potential placement of a ventriculostomy as a means to assess ICP and potentially treat
with CSF drainage.
If elevated ICP is present, therapy should be directed to maintain ICP < 20 mm Hg. Interventions should be used only when ICP is sustained above 20
mm Hg for more than 5 minutes. Transient physiologic elevations in ICP may be observed in the setting of coughing, movement, suctioning, or
ventilator asynchrony and should not be targeted by therapy. During periods of elevated ICP, it is important to maintain adequate mean arterial
pressure (MAP) to ensure adequate cerebral perfusion pressure (CPP; which is equal to MAP minus ICP).
GENERAL MANAGEMENT OF ELEVATED ICP
1. Blood pressure control
Therapy with vasopressors should be targeted to maintain an adequate CPP (CPP = MAP – ICP), typically > 60 mm Hg. Hypertension should generally
only be treated when CPP > 120 mm Hg. CPP < 50 mm Hg is associated with cerebral ischemia.
2. Head position
Patients with elevated ICP should be positioned with the head elevated 30 degrees above the heart while maintaining the neck in neutral position
without excessive flexion or rotation to maximize intracranial venous outflow.
3. Removal of CSF
When hydrocephalus is discovered, a ventriculostomy may be used to reduce intracranial CSF volume and secondarily ICP. Controlled drainage of CSF
at a rate of approximately 1-2 mL/min until the goal ICP is reached (ICP < 20 mm Hg) or until CSF is no longer easily obtained is recommended. CSF
drainage via an intrathecal lumbar drain is generally contraindicated in the setting of elevated ICP due to the risk of inducing herniation through a
pressure differential.
4. Hyperventilation
The use of mechanical ventilation to lower PaCO2 to a goal of 26-30 mm Hg induces cerebral vasoconstriction, decreasing the cerebral blood volume
and rapidly reducing ICP. The effect of hyperventilation on ICP typically lasts for a period of hours prior to metabolic compensation. Therapeutic
hyperventilation should be used only as an emergent intervention to temporarily control ICP and be replaced by other therapy modalities.
HYPEROSMOTIC THERAPY
1. Mannitol
Osmotic diuretics reduce brain volume by creating an osmotic gradient from the brain parenchyma to the intravascular space, thus drawing free water
from the parenchyma. It is also known to decrease blood viscosity by altering red blood cell rheology for more effective oxygen delivery andPage
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free radical scavenger. Mannitol is prepared in a 20% solution and can be given as a bolus of 1-1.5 g/kg. Mannitol can be used in serial doses at
intervals of every 6 hours at a reduced dosage of 0.25-0.5 g/kg as needed for continued elevated ICP. The onset of action is within minutes, and the
duration of effect varies widely from 4-24 hours. Serial measurements of serum sodium, serum osmolality, and renal function are necessary to prevent
hyperventilation should be used only as an emergent intervention to temporarily control ICP and be replaced by other therapy modalities.
HYPEROSMOTIC THERAPY
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1. Mannitol
Osmotic diuretics reduce brain volume by creating an osmotic gradient from the brain parenchyma to the intravascular space, thus drawing free water
from the parenchyma. It is also known to decrease blood viscosity by altering red blood cell rheology for more effective oxygen delivery and acts as a
free radical scavenger. Mannitol is prepared in a 20% solution and can be given as a bolus of 1-1.5 g/kg. Mannitol can be used in serial doses at
intervals of every 6 hours at a reduced dosage of 0.25-0.5 g/kg as needed for continued elevated ICP. The onset of action is within minutes, and the
duration of effect varies widely from 4-24 hours. Serial measurements of serum sodium, serum osmolality, and renal function are necessary to prevent
overdosage and end-organ failure. Contraindications to the use of mannitol include serum sodium > 150 mEq, serum osmolality > 320 mOsm, or
evidence of evolving acute tubular necrosis. In addition, mannitol may induce hypotension and subsequently decrease cerebral perfusion. Mannitol
should be used with care in patients with acute or chronic renal disease.
2. Hypertonic saline
Bolus dosing of hypertonic saline (typical tonicity ranging from 1.8%-23.4%) can acutely lower ICP. The typical volume of hypertonic saline varies widely
depending on tonicity, ranging from 30 mL of 23.4% to 1 L of 1.8%. Continuous infusion of hypertonic saline (1.8%-3%) to maintain hypernatremia may
also be effective in controlling ICP. A meta-analysis of multiple clinical trials comparing the efficacy of mannitol to hypertonic saline for management of
elevated ICP from a variety of causes (eg, traumatic brain injury, stroke, tumors) found that hypertonic saline appeared to have greater efficacy in
reducing elevated ICP, but clinical outcomes were not examined. Hypertonic saline should generally not be administered through a peripheral
intravenous catheter.
3. Fluid management
Typically, patients with elevated ICP should be kept euvolemic and normo- to hyperosmolar. Administration of free water and hypotonic solutions
should be strictly avoided, and instead, isotonic fluids should be used in all maintenance fluids and infusions. Serum sodium levels should be closely
monitored, and hyponatremia should be appropriately corrected.
4. Sedation
Maintenance of adequate sedation can decrease ICP by reducing cerebral metabolic demand, ventilator asynchrony, and the sympathetic responses of
hypertension and tachycardia. Adequate sedation typically requires the establishment of a secure airway. Infusion of propofol is often the drug of
choice for sedation because it can be rapidly titrated, thus allowing for frequent neurologic assessments. Adequate pain control, often using fentanyl
bolus or infusion, should be maintained while patients are chemically sedated.
5. Fever
Fever increases brain metabolism, which increases cerebral blood flow and thus elevates ICP. Additionally, fever has been demonstrated to worsen
brain injury in animal models. Therefore, aggressive treatment of fever, including acetaminophen and cooling, is recommended in patients with
increased ICP.
6. Antiepileptic therapy
Seizures, either convulsive or nonconvulsive, increase cerebral metabolism, resulting in ICP elevation. Aggressive treatment of seizures with
antiepileptic therapy or infusion of anesthetics and continuous EEG monitoring are warranted. However, prophylactic antiepileptic therapy is not
indicated for direct treatment or prophylaxis of elevated ICP, although it may be reasonable to consider in cases of high-risk structural lesions such as
tumor or hemorrhage within supratentorial cortical locations or lesions in contact with the cerebral cortex.
7. Glucocorticoids
Glucocorticoids, typically dexamethasone, are reserved for the management of elevated ICP due to vasogenic edema, most often secondary to
intracranial neoplasm. Typically, dexamethasone is dosed every 6-24 hours at doses ranging from 2-6 mg. The use of glucocorticoids has been
associated with a worse outcome in a large randomized clinical trial in traumatic brain injury and is not recommended. Glucocorticoids are not
considered to be useful in the management of cerebral infarction or intracranial hemorrhage.
8. Barbiturates
The use of barbiturates to control ICP is based on the drug’s ability to dramatically reduce brain metabolism and secondarily cerebral bloodPage
Chapter 38: Neurosurgery, Timothy R. Smith flow.9 / 93
Pentobarbital is most commonly used, with a loading dose of 5-20 mg/kg as a bolus, followed by 1-4 mg/kg/h. The barbiturate infusion is titrated
based on assessment of ICP and CPP and tolerance of side effects. Continuous EEG monitoring is generally used, with titration to an EEG burst
Glucocorticoids, typically dexamethasone, are reserved for the management of elevated ICP due to vasogenic edema, most often secondary to
intracranial neoplasm. Typically, dexamethasone is dosed every 6-24 hours at doses ranging from 2-6 mg. The use of glucocorticoids has been
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associated with a worse outcome in a large randomized clinical trial in traumatic brain injury and is not recommended. Glucocorticoids are not
considered to be useful in the management of cerebral infarction or intracranial hemorrhage.
8. Barbiturates
The use of barbiturates to control ICP is based on the drug’s ability to dramatically reduce brain metabolism and secondarily cerebral blood flow.
Pentobarbital is most commonly used, with a loading dose of 5-20 mg/kg as a bolus, followed by 1-4 mg/kg/h. The barbiturate infusion is titrated
based on assessment of ICP and CPP and tolerance of side effects. Continuous EEG monitoring is generally used, with titration to an EEG burst
suppression pattern indicating appropriate suppression of cerebral metabolism. Barbiturate therapy is complicated and fraught with complications,
particularly hypotension, adynamic ileus, reduced mucociliary clearance of the airway, and increased risk of infections. In general, the use of
barbiturates is reserved for elevated ICP refractory to all other treatment modalities.
9. Therapeutic hypothermia
The use of hypothermia to treat elevated ICP has been controversial for decades, and its use is currently not recommended in cases of trauma.
However, out-of-hospital cardiac arrest patients have been shown to have improved neurologic outcomes following targeted therapeutic hypothermia,
such that it is now part of the American Heart Association’s guidelines. Hypothermia decreases cerebral metabolism and secondarily reduces cerebral
blood volume and ICP. When used, hypothermia is achieved by whole-body cooling using surface or intravascular cooling devices to a goal of 32-34°C.
Some studies have demonstrated significant adverse effects, including cardiac arrhythmias and severe coagulopathy. Given the uncertainty
surrounding appropriate use of therapeutic hypothermia in patients with elevated ICP, this treatment should be limited to post–cardiac arrest patients
or those with intracranial hypertension refractory to other therapies.
10. Paralysis
Sustained paralytic use may be employed as an end-of-the-line medical measure to promote ventilator synchrony, prevent brisk bucking and straining,
and reduce overall systemic metabolism, allowing for increased oxygen delivery to the cerebrum. The literature generally does not support the use of
paralysis, as it has been shown to cause a host of complications and prolong ICU length of stay, without clear benefit.
11. Decompressive craniectomy
Decompressive craniectomy is the surgical removal a large portion of the cranial vault to allow for the edematous intracranial contents to expand into
the newly enlarged and soft compartment below the scalp to reduce ICP. At the time of the procedure, any mass lesion (neoplasm or hematoma) is also
removed. Decompressive craniectomy is often considered as a last resort for ICP management. There exists evidence that it decreases mortality but
increases the number of patients with poor neurologic outcomes in cases of severe traumatic brain injury with refractory ICP. It has also shown
potential benefit in healthy younger stroke patients suffering from malignant middle cerebral artery infarction.
Aslaner MA, Boz M, Celik A, et al: Etiologies and delirium rates of elderly ED patients with acutely altered mental status: a multicenter prospective
study. Am J Emerg Med. 2017;35(1):71–76. [PubMed: 27765479]
Bhatia A, Gupta AK: Neuromonitoring in the intensive care unit. I. Intracranial pressure and cerebral blood flow monitoring. Intensive Care Med .
2007;33:1263–1271. [PubMed: 17522844]
Diedler J, Sykora M, Blatow M, et al: Decompressive surgery for severe brain edema. J Intensive Care Med. 2009;24:168. [PubMed: 19321537]
Edwards P, Arango M, Balica L, et al: Final results of MRC CRASH, a randomised placebo-controlled trial of intravenous corticosteroid in adults with
head injury-outcomes at 6 months. Lancet. 2005;365:1957. [PubMed: 15936423]
Kamel H, Navi BB, Nakagawa K, et al: Hypertonic saline versus mannitol for the treatment of elevated intracranial pressure: a meta-analysis of
randomized clinical trials. Crit Care Med. 2011;39:554. [PubMed: 21242790]
The Brain Trauma Foundation; American Association of Neurological Surgeons; Congress of Neurological Surgeons; Joint Section on Neurotrauma
and Critical Care. Guidelines for the management of severe traumatic brain injury. VI. Indications for intracranial pressure monitoring. J Neurotrauma.
2007;24:S37–S44. [PubMed: 17511544]
IMAGING OF THE CENTRAL NERVOUS SYSTEM
The Brain Trauma Foundation; American Association of Neurological Surgeons; Congress of Neurological Surgeons; Joint Section on Neurotrauma
and Critical Care. Guidelines for the management of severe traumatic brain injury. VI. Indications for intracranial pressure monitoring. J Neurotrauma.
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2007;24:S37–S44. [PubMed: 17511544]
IMAGING OF THE CENTRAL NERVOUS SYSTEM

INTRODUCTION
Imaging has become central to medical care over the past 25 years and is one of the fastest-growing components of medical care expenditures in this
country. In no field of medicine has imaging made more of an impact than in the neurosciences. The ability to demonstrate anatomy and pathology
noninvasively and treat many pathologic central nervous system (CNS) processes using minimally invasive techniques has grown immensely with the
recent improvements in imaging technology.
Forty years ago, the only way to image the CNS was to replace CSF with contrast material (dye) or air via a lumbar puncture and take x-rays—a difficult
technique called pneumoencephalography. Normal and abnormal CNS structures could be crudely outlined in this manner. Pneumoencephalography
was abandoned in the late 1970s with the development of CT. Injecting dye directly into major blood vessels of the neck (cerebral angiography) has
been available for three-quarters of a century and allows exquisite delineation of intrinsic vascular pathology but still does little to directly visualize the
brain or spinal cord. Although angiography is still performed today for both diagnostic and therapeutic purposes, the catheter is now placed via
femoral or radial arterial cannulation instead of directly into a neck vessel.
With the advent of CT scanning in the early 1970s, direct visualization of the brain was finally possible, although resolving normal brain parenchymal
structures and some pathologic processes remained difficult. MRI first became available for clinical use in the early 1980s and has been the standard
for evaluation of most CNS processes since the mid-1980s.
CT and MRI have continued to mature through the 1990s and into the 21st century. In addition to the introduction of lower radiation dose scanner
capabilities on the most recent generation (since the late 2000s) of CT scanners, rapid scanning techniques on such scanners (multislice or
multidetector CT scanners) now allow for an increasing amount of data collection and resolution in a shorter period of time. Similarly, such state-of-
the-art scanners can be used to generate models of the major blood vessels of the brain (CT angiograms), lessening the need for more invasive
intravascular catherization in some patients.
MRI advances have been equally as dramatic, particularly with the release of higher field strength magnetic resonance (MR) scanners (3.0 T) for clinical
use in 2005. For example, the sensitivity of some of the newer MRI scanning techniques enables identification of physiologic changes in the brain
minutes after ischemia occurs, a time frame that can potentially allow for pharmacologic or interventional procedures that might improve clinical
outcomes (eg, stroke). Functional MRI can be used to assess eloquent areas of the brain near pathologic lesions (eg, tumors) that should be avoided
during surgery. MR spectroscopy (MRS) can identify lesion metabolites that may help discriminate among pathologic processes (eg, tumor, radiation
necrosis, ischemia, inflammation, or infection); however, its true clinical utility is still under debate. MRS can also potentially identify metabolites
accumulating in the brain in patients with congenital metabolic disorders. MR angiography can be used to image and form three-dimensional
reconstructions of blood vessels for evaluation without injection of dye or the use of ionizing radiation.
Endovascular angiography has been available in various forms for over 80 years and has continued to evolve rapidly over past decade. Initially used as
a diagnostic tool, the field of interventional neuroradiology now employs a variety of multimodal therapies and continues to experience significant
growth, as it exists at the cross-section of multiple disciplines, including radiology, neurology, and neurosurgery. Neuro-interventionalists are now
able to visualize and treat vascular abnormalities such as cerebral aneurysms or arteriovenous malformations through a catheter as an adjunct to, or
even in lieu of, open surgery. At this time, more than 70% of cerebral aneurysms treated in the United States are currently managed endovascularly,
avoiding the need for craniotomy in some patients. Given the less invasive nature yet generally durable outcomes of endovascular treatment, it
remains an area of increasing innovation and has been widely adopted across the globe.
BASIC CENTRAL NERVOUS SYSTEM IMAGING TECHNIQUES
1. Plain radiographs (ie, x-rays) are relatively inexpensive, universally available, and sensitive to detecting osseous abnormalities such as fractures or
gross destructive lesions (Figure 38–2). The main disadvantage of plain x-rays is that soft tissues (eg, brain and spinal cord) and many pathologic
processes (eg, hemorrhage, infarctions, tumors, abscesses, herniated discs) cannot be detected. Even many intrinsic osseous lesions are difficult
to delineate. In fact, until 30%-50% of bone marrow trabeculae are replaced by a pathologic process (eg, tumor, infection), no osseous abnormality
is seen on a plain radiograph.
Another limitation of plain radiographs is that all structures are superimposed on a single two-dimensional image as the x-ray beam passes
through the entire head or spine (as opposed to the multiple “slices” generated on CT and MRI scans).
1. Plain radiographs (ie, x-rays) are relatively inexpensive, universally available, and sensitive to detecting osseous abnormalities such as fractures or
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gross destructive lesions (Figure 38–2). The main disadvantage of plain x-rays is that soft tissues (eg, brain and spinal cord) and many pathologic
processes (eg, hemorrhage, infarctions, tumors, abscesses, herniated discs) cannot be detected. Even many intrinsic osseous lesions are difficult
to delineate. In fact, until 30%-50% of bone marrow trabeculae are replaced by a pathologic process (eg, tumor, infection), no osseous abnormality
is seen on a plain radiograph.
Another limitation of plain radiographs is that all structures are superimposed on a single two-dimensional image as the x-ray beam passes
through the entire head or spine (as opposed to the multiple “slices” generated on CT and MRI scans).
There is a limited role for plain radiographs during the evaluation of CNS disease as the soft tissue structures within the cranium and spinal canal
are not imaged. However, x-rays offer a cost-effective and efficient means to assess spinal column deformity or misalignment and are often used to
evaluate for motion between vertebral bodies of the spinal column as they can be performed in a relatively dynamic fashion (ie, lateral
flexion/extension/standing radiographs of the cervical or lumbar spine) compared to CT or MRI imaging. Furthermore, they can be used in the
postoperative setting to evaluate placement of internal spinal hardware.
2. Ultrasound of the CNS is predominantly used in the neonatal period through the anterior fontanelle as a noninvasive and radiation-free method by
which to screen for intraventricular hemorrhage, abnormal ventricular morphology, or gross underlying parenchymal pathology. It is particularly
useful for trending ventricular dimensions in infants suffering from hydrocephalus or for viewing the conus medullaris in patients younger than 3
months of age. In addition, prenatal ultrasound as a means of early screening for brain or spinal anomalies is routinely employed. Ultrasound
technology also has growing applications in adult patients with neurologic disease, both in and out of the operating room. Intraoperative
ultrasound has grown in popularity with neurosurgeons because it offers “live” imaging that can be particularly useful for localizing pathology and
evaluating extent of lesion resection and can also be used to help position ventricular catheters during surgery.
Adult patients suffering from a ruptured cerebral aneurysm are at increased risk for vasospasm and subsequent stroke, and transcranial Doppler
examinations to diagnose this disease may be performed by scanning through the thin temporal bone. A specially trained ultrasonographer
provides bedside measurements of the velocities of major intracranial arteries (ie, anterior, middle, and posterior cerebral arteries) and compares
them to a control, often the internal carotid artery. This provides a noninvasive means to assess for cerebral artery vasospasm in high-risk patients.
In addition, the ultrasonographer may perform a lengthier study to evaluate for “hits” (ie, embolic events) that could be causing symptoms of
transient ischemic attack or stroke.
3. CT scanning with or without intravenous administration of iodinated contrast material is performed using a thin fan-like band of x-rays (ionizing
radiation) that are generated by an x-ray–emitting tube that rotates around the patient on the order of seconds. The resultant image, which
represents a “slice” of tissue, can be obtained with images as thin as 1 mm. “Reformatted” submillimeter images or three-dimensional models can
also be generated in nearly any plane (eg, sagittal, coronal, off-axis) by the CT computer software using axial CT scan data in any plane (eg, sagittal,
coronal, off-axis,). Three-dimensional images can also be created in this manner. One notable risk of iodinated contrast administered during CT
angiograms (CTA) is the risk of causing contrast-induced neuropathy, the third most common cause of acute kidney injury in the hospitalized
patient.
CT demonstrates most osseous abnormalities better than any other imaging test with the possible exception of nondisplaced, nondistracted
fractures, which are still often better seen on plain x-rays (Figure 38–3). Soft tissue lesions can also be detected with much better sensitivity than
plain x-rays. However, resolving some similar, but not identical, normal soft tissues from one another, delineating normal from pathologic soft
tissue, and evaluating certain areas of the brain that are limited by scanning artifacts can still be difficult with CT scanning, which is why CT remains
inferior to MRI for evaluation of most brain and spinal canal abnormalities.
When discussing CT images, the terms density and attenuation refer to the same process, namely absorption of the x-ray beam. Areas of increased
“density” have greater “attenuation” of the x-ray beam and result in a brighter white appearance on the CT image (eg, bones, iodine-based contrast
material, acute blood, areas of calcification, and some foreign bodies). Areas of lower density have lower attenuation because they absorb less of
the x-ray beam as it passes through the patient, resulting in darker areas on the CT scan image (eg, fluid in the ventricles, gas, and fat). CT scans are
exceptionally useful for rapid assessment of the intracranial vault, such as in situations of neurologic decline that could be due to a structural cause
(eg, hemorrhage, tumor, stroke), with the caveat that acute strokes may be difficult to detect on CT scan and are more readily diagnosed based on
clinical assessment and/or MRI. CT scans are also often obtained on an elective basis for patients who will be undergoing certain types of
neurologic, sinus, or temporal bone surgery.
The addition of contrast agents to CT scans, CTA, has proven to be exceptionally useful in regard to evaluating extra- and intracerebral vasculature.
CTA provides a useful, noninvasive alternative to catheter angiography for assessment of vasculature, although it lacks the dimension of time,
which can be useful in assessment of certain pathologies such as dural arteriovenous fistulas or arteriovenous malformations. More recently,
advances in technology have allowed for the development of the dynamic CTA (dCTA). This advanced imaging modality allows for the contrast
bolus and scan to be timed such that the slices acquired have a temporal component to them much like a traditional catheter angiogram.
Chapter 38: Neurosurgery, Timothy R. Smith Although
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not yet widely available, dCTA is another step toward a noninvasive means to replacing diagnostic cerebral angiograms.
4. Myelography involves performing a lumbar subarachnoid puncture (or a lateral C1-2 subarachnoid puncture) and injecting less than 1 oz of
neurologic, sinus, or temporal bone surgery.
The addition of contrast agents to CT scans, CTA, has proven to be exceptionally useful in regard to evaluating extra-Access Provided by:
and intracerebral vasculature.
CTA provides a useful, noninvasive alternative to catheter angiography for assessment of vasculature, although it lacks the dimension of time,
which can be useful in assessment of certain pathologies such as dural arteriovenous fistulas or arteriovenous malformations. More recently,
advances in technology have allowed for the development of the dynamic CTA (dCTA). This advanced imaging modality allows for the contrast
bolus and scan to be timed such that the slices acquired have a temporal component to them much like a traditional catheter angiogram. Although
not yet widely available, dCTA is another step toward a noninvasive means to replacing diagnostic cerebral angiograms.
4. Myelography involves performing a lumbar subarachnoid puncture (or a lateral C1-2 subarachnoid puncture) and injecting less than 1 oz of
iodinated contrast material to opacify the spinal canal subarachnoid space, which results in contrast filling of the spaces around the spinal cord.
This is particularly useful for evaluating for areas of cord or nerve root compression from extradural lesions such as epidural tumors, hematomas,
bone spurs, abscesses, misplaced spinal hardware, or herniated disks. In addition, some intramedullary lesions, such as spinal cord tumors, may
be picked up on myelography.
MRI has replaced myelography as the primary imaging modality for evaluating the contents of the spinal canal. At this time, it is mainly reserved for
situations when MRI is not possible to obtain (eg, history of metal foreign body or MR-incompatible cardiac pacemakers) or produces suboptimal
images (eg, spinal hardware).
Modern myelography is essentially always followed immediately by CT scanning to better delineate relationships of pathologic processes to the
subarachnoid space.
5. MRI is an imaging technique that does not use ionizing radiation.
The physics of creating an MR scan are quite complex. Briefly, a patient is placed in a strong magnetic field (30,000× greater than that of the earth’s
magnetic field). Radiofrequency pulses are transiently (milliseconds) applied to the patient to briefly perturb the water molecules within the patient
by raising them to a slightly higher energy level (quantum mechanical model). After turning off the radiofrequency pulse, these water molecules
rapidly return to their respective baseline states and, in the process, release their recently absorbed energy. The rate at which these perturbed
water molecules return to their respective baseline states can be measured by using extremely sensitive receiver coils in the MR scanner. As the
rates at which these molecules return to their respective baseline states varies by local magnetic environment (eg, the local magnetic environment
is different in the ventricular system as opposed to the lentiform nucleus, white matter, the eyeball, muscles, tumors, etc), these detectable
differences among tissues can be localized in three-dimensional space and used to generate an image.
MRIs are obtained that highlight different kinds of magnetic field differences between tissues. In general, most MR studies include T1-weighted and
T2-weighted scans as part of the overall evaluation of the patient. MRI scans take longer to perform than CT scans in part because T1-weighted and
T2-weighted scans and any additional sequences must be obtained separately. T1-weighted images are distinguishable by the black appearance
(absence of MR signal) of the CSF over the surface of the brain and in the ventricles. In contrast, on a T2-weighted scan, the CSF over the surface of
the brain and in the ventricles appears bright white (Figure 38–4).
In general, T1-weighted scans are best for delineating cerebral anatomy and areas of contrast enhancement, which occur in many tumors. T2-
weighted scans are exquisitely sensitive to subtle changes in water concentrations (in both normal and pathologic tissues). This makes them ideal
for assessment of many pathologic processes such as stroke, tumor, infection, or edema. These subtle changes manifest as increased signal
(brighter) on T2-weighted scans.
Fluid-attenuated inversion recovery (FLAIR) scans are a form of T2-weighted sequence in which normal fluid (eg, in the ventricles and subarachnoid
spaces) has no signal (ie, appears black) and, therefore, subtle pathologic process (which still appear bright on FLAIR images, similar to that seen
on standard T2-weighted scans) are easier to identify.
Diffusion-weighted imaging (DWI) is obtained by measuring random motion of water molecules within a defined area (voxel) of tissue. In general,
highly cellular tissue and tissue with significant cellular swelling (such as seen after stroke) exhibit lower diffusion coefficients, often referred to as
diffusion restriction. Tissue “restriction” can represent such entities as hypercellular neoplasms, ischemic stroke, diffuse axonal injury, cerebral
abscesses, or demyelinating disease.
Susceptibility-weighted imaging (SWI) is a sequence that exhibits exquisite sensitivity to compounds that distort the local magnetic field, making
them exceptionally useful for detecting blood products or calcium within the brain. In certain vascular pathologies that demonstrate a history of
recurrent hemorrhage, the SWI technique offers a sensitive means by which to screen for these lesions. The most notable example of such lesions
is cavernous malformation.
Gadolinium-based contrast agents may also be delivered via intravenous injection to enhance the appearance of normal anatomic and vascular
structures, as well as pathologic lesions within the brain (primarily neoplasms or infections). Enhancement pattern may differ significantly
Chapter 38: Neurosurgery, Timothy R. Smith Pageamong
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various neoplasms, suggesting certain diagnoses. In patients with poor renal function (glomerular filtration rate < 30 mL/min), gadolinium is
contraindicated due to the risk of developing nephrogenic systemic fibrosis.
Susceptibility-weighted imaging (SWI) is a sequence that exhibits exquisite sensitivity to compounds that distort the local magnetic field, making
them exceptionally useful for detecting blood products or calcium within the brain. In certain vascular pathologies that demonstrate a history of
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recurrent hemorrhage, the SWI technique offers a sensitive means by which to screen for these lesions. The most notable example of such lesions
is cavernous malformation.
Gadolinium-based contrast agents may also be delivered via intravenous injection to enhance the appearance of normal anatomic and vascular
structures, as well as pathologic lesions within the brain (primarily neoplasms or infections). Enhancement pattern may differ significantly among
various neoplasms, suggesting certain diagnoses. In patients with poor renal function (glomerular filtration rate < 30 mL/min), gadolinium is
contraindicated due to the risk of developing nephrogenic systemic fibrosis.
MRI best delineates soft tissues both intrinsic and immediately extrinsic to the brain, can be performed in any plane (including nonorthogonal
planes), and has no known adverse effects at the field strengths used for clinical studies. In addition to better demonstrating tissues than CT, MRI is
not limited by some of the artifacts that limit evaluation by CT, particularly in the posterior fossa and spinal canal, where CT artifact can be
problematic. Although not ideal for directly visualizing dense osseous structures (where fractures, cortical erosions, and degenerative changes
often occur), MR excels at detecting intrinsic (ie, bone marrow) osseous abnormalities (eg, primary bone tumors, metastatic neoplasms, lymphoma,
vascular anomalies, osteomyelitis).
6. Cerebral angiography is performed by directly placing a catheter into the femoral or radial artery, passing it in a retrograde manner up into either a
vertebral or carotid artery, and then directing it further cephalad until it reaches the intracranial vasculature (Figure 38–5). The catheter is moved
using intermittent fluoroscopic guidance with small amounts of dye injected at selected intervals to confirm the location of the catheter. When in
position, larger amounts of dye are injected, and serial plain digital x-rays are rapidly exposed as the dye passes through the intracranial vessels in
the distribution of the injected blood vessel. Hundreds of images might be obtained during a single injection of contrast material. This procedure is
associated with a 0.1%-0.5% chance of causing a symptomatic stroke. Embolic events that are clinically silent occur on an even more regular basis.
This technique best delineates intrinsic blood vessel pathology such as arterial stenosis, aneurysms, arteriovenous malformations, fistulas,
moyamoya disease, vasculitis, occluded vessels, and other intrinsic vascular disorders. It can be used to inject dye or medications into specific
vascular territories for diagnostic or therapeutic purposes. Catheters can be used to deliver a wide variety of therapeutic devices such as
prothrombotic coils and intravascular stents for treatment of aneurysms or intravascular glue for embolization of vascular malformations. Balloon
angioplasty or stenting can be used to maintain vessel patency in areas of focal stenosis. There are even mechanical or suction-based methods for
retrieving intravascular embolic clot for treatment of patients experiencing stroke.
Endovascular (catheter) angiography has come a long way since its inception in the early 1900s; however, it remains expensive and has a non-zero
risk of complications. It also requires complex equipment and is performed by highly trained personnel, often at more advanced care centers. In
addition, operator skill continues to play an important role in both diagnostic and therapeutic outcomes. Even with advances in x-ray technology,
there remains significant exposure to radiation over the lifetime of the operator. There are currently several alternatives to catheter angiography to
evaluate for vascular lesions, such as ultrasound (mainly to assess carotid stenosis), CTA, or MR angiography; each is useful for particular
situations or pathologies (Figure 38–6). Despite these evolving diagnostic modalities, catheter angiography remains the gold standard for
intravascular imaging given its high resolution and temporal component.
7. Radionuclide imaging (scintigraphy) includes positron emission tomography (PET) and single-photon emission CT (SPECT) scans, both of which
are molecular imaging techniques. Unlike other imaging techniques, these imaging modalities provide information beyond the structural
appearance of normal and pathologic tissues. These imaging methods provide physiologic information on the functional state of tissues.
Low doses of radiotracers (positron emitters for PET scanning and single-photon–emitting isotopes for SPECT scanning) are used to label
molecules or pharmacologic agents to image molecular interactions of biologic processes in vivo. The nanomolar concentration of the radiotracer
allows in vivo assessment of biologic processes without interfering with the process itself.
PET and SPECT cameras are able to detect, localize, and quantify regional distribution of radioactivity within the brain. Reconstruction techniques
similar to those used in CT (eg, filtered backprojection and iterative reconstruction) yield two- and three-dimensional images of the brain.
The main advantage of PET over SPECT imaging is that PET uses “coincidence” detection, which is the ability to detect the simultaneous emission of
two gamma rays that are generated when positrons annihilate when encountering negative electrons. Practically, this results in the greater spatial
resolution of PET compared to SPECT. Both techniques can be used to study the same biologic processes.
The biologic significance of measured radioactivity on PET and SPECT scans depends on the biological function of the molecule that is attached to
the radioisotope. For example, radioligands are available to measure cerebral blood flow, blood–brain barrier permeability, neurotransmitter
synthesis, enzyme activity, receptor density, glucose metabolic rates, and gene expression, among other physiologic processes. Analysis of regional
brain functions can be more specific by performing imaging both before and after specific pharmacologic interventions, specialized motor or
mental tasks (so-called “activation” studies), or therapeutic interventions, such as stem cell or gene therapy.
PET or SPECT imaging can also be used in the clinical arena for localization of epileptogenic foci, investigating dementing disorders, distinguishing
between tumor recurrence and radionecrosis, and providing a chronologic record of disease progression (or response to therapy).
resolution of PET compared to SPECT. Both techniques can be used to study the same biologic processes.
The biologic significance of measured radioactivity on PET and SPECT scans depends on the biological function of the molecule that is attached to
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the radioisotope. For example, radioligands are available to measure cerebral blood flow, blood–brain barrier permeability, neurotransmitter
synthesis, enzyme activity, receptor density, glucose metabolic rates, and gene expression, among other physiologic processes. Analysis of regional
brain functions can be more specific by performing imaging both before and after specific pharmacologic interventions, specialized motor or
mental tasks (so-called “activation” studies), or therapeutic interventions, such as stem cell or gene therapy.
PET or SPECT imaging can also be used in the clinical arena for localization of epileptogenic foci, investigating dementing disorders, distinguishing
between tumor recurrence and radionecrosis, and providing a chronologic record of disease progression (or response to therapy).
Finally, the same SPECT radioligands that are used for cerebral blood flow imaging (eg, Tc-99m HMPAO) can also be used to diagnose brain death.
Radioligands such as In-111 DTPA can be used intrathecally for radioisotope cisternograms for the clinical evaluation of patients with
hydrocephalus or suspected CSF leaks. Radioisotope studies can also be performed to evaluate for leakage of CSF from ventricular shunts.
Figure 38–2.
Lateral plain radiograph (x-ray) of the skull (ionizing radiation). Note the excellent delineation of the osseous structures but poor delineation of soft
tissues; specifically, no portion of the brain is imaged. In addition, this study is not tomographic (ie, is not a slice), and therefore, both the left and right
sides of the head are superimposed.
Figure 38–3.
Computed tomography scan (ionizing radiation). This image represents a single section (“slice”) through the head (ie, it is a tomographic image; the
left and right sides of the head can be separately delineated). The brain is directly imaged, although resolving differences in intracranial structures can
be difficult (eg, resolving gray and matter structures).
Figure 38–3.
Computed tomography scan (ionizing radiation). This image represents a single section (“slice”) through the head (ie, it is a tomographic image; the
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left and right sides of the head can be separately delineated). The brain is directly imaged, although resolving differences in intracranial structures can
be difficult (eg, resolving gray and matter structures).
Figure 38–4.
Magnetic resonance scan (no ionizing radiation). Similar to computed tomography (CT), this image represents a single section (“slice”) through the
head. The white matter and gray matter structures are more easily resolved than on a CT scan, and even some gray matter structures can be resolved
(eg, putamina and globus pallidae).
Figure 38–4.
Magnetic resonance scan (no ionizing radiation). Similar to computed tomography (CT), this image represents a single section (“slice”) through the
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head. The white matter and gray matter structures are more easily resolved than on a CT scan, and even some gray matter structures can be resolved
(eg, putamina and globus pallidae).
Figure 38–5.
Angiogram (ionizing radiation). A catheter was placed in the right carotid artery in the neck and dye was injected while imaging was performed in a
front-to-back projection of the right side of the head. The bones are then digitally “subtracted” from the image. The exquisite delineation of the blood
vessels of the right carotid territory are demonstrated but no intra-axial (ie, brain soft tissues) structures.
Figure 38–5.
Angiogram (ionizing radiation). A catheter was placed in the right carotid artery in the neck and dye was injected while imaging was performed in a
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front-to-back projection of the right side of the head. The bones are then digitally “subtracted” from the image. The exquisite delineation of the blood
vessels of the right carotid territory are demonstrated but no intra-axial (ie, brain soft tissues) structures.
Figure 38–6.
Aneurysm demonstrated by multiple imaging techniques. A : Catheter angiogram (ionizing radiation). B : Computed tomography (CT) angiogram
(ionizing radiation). C : Magnetic resonance (MR) angiogram (no ionizing radiation). Note that the catheter angiogram (A; an imaging modality that is
associated with a risk of causing a stroke) best defines this anterior communicating artery region aneurysm (arrow). However, the aneurysm is still well
seen on the CT angiogram (B), an imaging technique that is not associated with any risk of causing a stroke, and is also well seen on the MR angiogram
(C), an imaging technique that is not associated with any risk of causing a stroke and does not employ ionizing radiation.
(ionizing radiation). C : Magnetic resonance (MR) angiogram (no ionizing radiation). Note that the catheter angiogram (A; an imaging modality that is
associated with a risk of causing a stroke) best defines this anterior communicating artery region aneurysm (arrow). However, the aneurysm is still well
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seen on the CT angiogram (B), an imaging technique that is not associated with any risk of causing a stroke, and is also well seen on the MR angiogram
(C), an imaging technique that is not associated with any risk of causing a stroke and does not employ ionizing radiation.
ADVANCED CENTRAL NERVOUS SYSTEM APPLICATIONS OF MRI, CT, AND ANGIOGRAPHY
Magnetic Resonance Imaging
A. MR Angiography
MR data can be collected using software that images only moving substance. Extravascular stationary soft tissue does not generate any MR signal on
such studies, whereas moving blood or CSF will generate signal. Using these techniques, images of blood vessels can be created with a sufficient level
of detail that, in some cases, can avoid contrasted scans or formal endovascular catheter angiography (eg, demonstration of significant atherosclerotic
disease in the region of the carotid artery bifurcation or surveillance imaging of a known aneurysm). CSF flow evaluation can also be performed (eg, at
the craniovertebral junction in Chiari patients).
B. Diffusion MRI and Perfusion MRI
Using rapidly applied magnetic field gradients, random molecular motion of water molecules can be converted to images and can be presented as
“diffusion” MRIs. Rapidly applied gradients can also be used with or without contrast agent administration to assess cerebral blood perfusion.
The combination of diffusion and perfusion sequences is used primarily in the field of stroke neurology. Diffusion MRI is sensitive to early cerebral
of detail that, in some cases, can avoid contrasted scans or formal endovascular catheter angiography (eg, demonstration of significant atherosclerotic
disease in the region of the carotid artery bifurcation or surveillance imaging of a known aneurysm). CSF flow evaluationInternational Medical University
can also be performed (eg, at
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the craniovertebral junction in Chiari patients).
B. Diffusion MRI and Perfusion MRI
Using rapidly applied magnetic field gradients, random molecular motion of water molecules can be converted to images and can be presented as
“diffusion” MRIs. Rapidly applied gradients can also be used with or without contrast agent administration to assess cerebral blood perfusion.
The combination of diffusion and perfusion sequences is used primarily in the field of stroke neurology. Diffusion MRI is sensitive to early cerebral
ischemia, often on the order of minutes. In the setting of acute cerebral ischemia, MR diffusion imaging changes (which usually reflect acute
irreversible ischemic change) and MR perfusion imaging changes (which reflect cerebral blood flow) can be performed. The MR perfusion and
diffusion images are then compared. As diffusion images usually represent permanent injury (infarction) and perfusion (blood flow) deficits are
potentially reversible, if an MR perfusion deficit is more extensive than an associated MR diffusion deficit, it is possible that an area of perfusion
abnormality without diffusion abnormality represents viable brain that is at risk of developing permanent injury but is not yet irreversibly injured. This
so-called “ischemic penumbra” may derive benefit from aggressive medical or interventional therapy. Alternatively, if a diffusion deficit (representing a
region of irreversible brain injury) is similar in extent to a perfusion deficit, this suggests that the ischemic changes are permanent and there is no
remaining “at-risk” penumbra of tissue that can be saved, and therefore, aggressive therapy may not be warranted.
C. MR Spectroscopy
Using standard MR hardware, MRS can be performed to evaluate brain metabolites. Normal brain tissue includes many metabolites, the most
important of which are N-acetyl-aspartate (NAA; which is found in normally functioning neurons and decreases with neuronal injury), choline (Cho; a
component of cell membranes that increases in any process that increases cellular turnover, such as tumors, acute infections, etc), and creatine (Cr; a
marker of cellular energy). Lactic acid can also be seen in ischemic cells (as a by-product of anaerobic glycolysis) but is not present in detectable
amounts on MRS in normal brain tissue. Of note, MRS is commonly used in an attempt to distinguish recurrent tumor from radiation necrosis in
postsurgical patients. In cases of recurrent tumor, Cho is elevated, whereas in radiation-induced change, NAA, Cho, and Cr are all low.
Computed Tomography
A. Biopsy
CT can be used for guidance to percutaneously biopsy or drain lesions that previously required an open surgical procedure, such as a spinal epidural
abscess or hematoma.
B. Perfusion CT
While rapidly infusing contrast material through a peripheral vein, using a high-speed multidetector CT scanner, data can be collected that reflects how
well different portions of the brain are being perfused. In some patients, there are areas of underperfused brain, which may manifest clinically with
transient symptoms (eg, transient ischemic attacks [TIAs]); these regions might benefit from revascularization before the patient suffers irreversible
injury (ie, stroke). Evaluations similar to brain MRI diffusion/perfusion imaging to assess for penumbra (see earlier) can also be performed.
C. CT Angiography
While rapidly infusing contrast material through a peripheral vein, using a high-speed multidetector CT scanner, data can be collected that can be
“reconstructed” with background data removed such that images similar to catheter angiograms can be created without risk of stroke. Although
intrinsic vascular detail is not as good as endovascular catheter angiography, in many patients, it is adequate to address the relevant clinical issue.
dCTA uses the same principles; however, it adds a temporal component for assessment of flow through vessels.
Angiography
A. Endovascular Coiling of Aneurysms
Small wires can be passed through an endovascular catheter and directly deposited into a saccular aneurysm where they form coils after release from
the catheter. An aneurysm can be filled with these wire coils, resulting in the complete obliteration of the patency of the aneurysm lumen, reducing or
eliminating the chance that such an aneurysm might rupture in the future and obviating the need for a craniotomy for placement of an aneurysm clip.
B. Stenting
Instead of open surgery to address a narrowed atherosclerotic or dissected blood vessel, affected blood vessels can be treated via an intravascular
catheter, which first uses a balloon to dilate the narrowed portion of the blood vessel and then releases a mesh stent to maintain the patency of the
newly dilated vessel. Stents can also be placed at the base of wide-necked aneurysms, allowing for the endovascular coiling of aneurysms that
otherwise would not be technically possible. This is referred to as stent-assisted coiling.
Small wires can be passed through an endovascular catheter and directly deposited into a saccular aneurysm where they form coils after release from
the catheter. An aneurysm can be filled with these wire coils, resulting in the complete obliteration of the patency of theInternational Medical University
aneurysm lumen, reducing or
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eliminating the chance that such an aneurysm might rupture in the future and obviating the need for a craniotomy for placement of an aneurysm clip.
B. Stenting
Instead of open surgery to address a narrowed atherosclerotic or dissected blood vessel, affected blood vessels can be treated via an intravascular
catheter, which first uses a balloon to dilate the narrowed portion of the blood vessel and then releases a mesh stent to maintain the patency of the
newly dilated vessel. Stents can also be placed at the base of wide-necked aneurysms, allowing for the endovascular coiling of aneurysms that
otherwise would not be technically possible. This is referred to as stent-assisted coiling.
C. Flow Diversion
In some instances, stent-like flow-diverting devices may be used, offering lower radial opening forces, increased metal coverage, and decreased
porosity compared to traditional endovascular stents. These devices are used to provide hemodynamic uncoupling of aneurysms, leading to eventual
thrombosis, with endothelialization of the flow-diverting stent to follow. To prevent intraluminal thrombus formation prior to endothelialization,
patients require dual antiplatelet agents (eg, aspirin and clopidogrel) to lower the risk of thrombotic complication. In addition, the altered flow and
eventual stent endothelialization pose a risk to daughter arteries that take off from the parent artery in a region covered by the flow diverter. However,
in practice, this has proven to have a relatively low incidence, occurring in less than 5% of patients. The indications for flow diversion are increasing
and often overlap with those of stenting and coiling. At this time, there remains significant provider-dependent variability in regard to the modality
chosen; however, as more data are accumulated, a more specific set of indications for each treatment should be reached.
D. Embolization
Although embolization has a significant history in cerebral angiography, its role is somewhat limited in the treatment of intracerebral pathology, and it
is mainly used as an adjunct to invasive open surgery. There are many types of embolic materials, such as coils, particles, foam, or microspheres that
may be used to occlude blood vessels (eg, those feeding a vascular tumor or the arterial inflow to an arteriovenous malformation) or thrombose
aneurysms. Embolization may be employed preoperatively in situations where the surgeon is concerned about a high likelihood of excessive bleeding
or to make the surgical procedure simpler and faster. Pure embolization of certain lesions still occurs, although the indications for such treatment
seem to become narrower as newer treatments are perfected and developed.
E. Treating Intraluminal Thrombus
An acute (within 6-8 hours of onset of symptoms) intraluminal occluding thrombus or thromboembolus can often be treated with intra-arterial
catheter placement within the blood clot and injection of a clot-lysing agent (eg, tissue plasminogen activator) to reestablish blood flow to an affected
vascular territory before permanent brain damage occurs. Alternatively, some intravascular clots can be captured and removed from the vascular
system using specialized clot retrieval catheters or suction devices.
Brinjikji W, Rabinstein AA, Nasr DM, et al: Better outcomes with treatment by coiling relative to clipping of unruptures intracranial aneurysms in the
United States. AJNR Am J Neuroradiol . 2011;32:1071–1075. [PubMed: 21511860]
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Hacke W, Kaste M, Bluhmki E, et al: Thrombolysis with alteplase 3 to 4.5 hours after acute ischemic stroke. N Engl J Med. 2008;359:1317–1329.
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Hopyan J, Ciarallo A, Dowlatshahi D, et al: Certainty of stroke diagnosis: incremental benefit with CT perfusion over non-contrast CT and CT
angiography. Radiology. 2010;255:142–153. [PubMed: 20308452]
McKinney AM, Palmer CS, Truwit CL, et al: Detection of aneurysms by 64-section multidetector CT angiography in patients acutely suspected of
having an intracranial aneurysm and comparison with digital subtraction and 3D rotational angiography. AJNR Am J Neuroradiol . 2008;29:594–602.
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Rajah G, Narayanan S, Rangel-Castilla L: Update on flow diverters for the endovascular management of cerebral aneurysms. Neurosurg Focus.
2017;42(6):E2.
Schellinger PD, Bryan RN, Caplan LR, et al: Evidence-based guideline: the role of diffusion and perfusion MRI for the diagnosis of acute ischemic
stroke: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2010;75:177–185.
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[PubMed: 18065510]
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Rajah G, Narayanan S, Rangel-Castilla L: Update on flow diverters for the endovascular management of cerebral aneurysms. Neurosurg Focus.
2017;42(6):E2.
Schellinger PD, Bryan RN, Caplan LR, et al: Evidence-based guideline: the role of diffusion and perfusion MRI for the diagnosis of acute ischemic
stroke: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2010;75:177–185.
[PubMed: 20625171]
CRANIOCEREBRAL TRAUMA
Pui Man Rosalind Lai, MD, & Timothy Smith, MD, PhD, MPH
KEY CONCEPTS
Traumatic brain injury (TBI) is a significant cause of morbidity and mortality in young adults.
Knowledge of the types of primary and secondary brain injury and their sequelae is important in the management of patients with severe TBI.
OVERVIEW
TBI is the leading cause of morbidity and mortality in the United States and is estimated to affect 2.5 million adults every year. Motor vehicle accident is
the most frequent cause of TBI in the developed world, accounting for 30%-50% of all serious head injuries. Traumatic falls and recreational injuries
account for 10%-15%, and inflicted injury or assaults account for 10%-20%. Although individuals of all ages can be affected by TBI, peak age is 15-24
years old, with a higher incidence in males. Head injury is frequent in polytrauma patients, and therefore, the basic knowledge of management for
these patients by emergency physicians and trauma surgeons is critical.
TBI can range from mild to severe based on clinical presentation. The majority of injuries (80%) are mild, including concussions, and do not require
hospitalization. Patients with moderate to severe injuries should be hospitalized and under close observation, as severe injuries can lead to lifelong
disability and death.
CLASSIFICATION
The injuries sustained in the setting of TBI can be divided into primary and secondary types. Primary injury occurs at the time of trauma and includes
deformative or concussive forces applied to the brain. Primary injuries include skull fractures, lacerations, and hemorrhages (which may take time to
fully accumulate). Generally, primary injury is considered irreversible and may be minimized with preventive measures, such as the use of a helmet.
Secondary injury is the brain’s biologic response to injury, which includes local inflammation, loss of regulation of cerebral blood flow (CBF), brain
edema, and global ischemia from increased ICP. At the cellular level, oxidative stresses from free radicals and apoptotic mechanisms are involved,
resulting in decreased CBF and tissue ischemia. At the macroscopic level, cerebral edema occurs through both cytotoxic (cell injury and swelling) and
vasogenic (incompetent vasculature) mechanisms. Because the skull is a rigid covering that limits space, any increase in amount of material, such as a
hematoma or edema, will result in increased ICP. In severe injuries, this increased pressure may result in brain material herniating out from its
compartment, causing clinical herniation syndromes, as discussed in the section on concussion in this chapter.
CLINICAL ASSESSMENT
The basic management of patients with severe brain injury can be divided into the following steps: initial resuscitation, primary neurologic survey,
immediate surgical management if necessary, and management of cerebral edema and increased ICP. Initial resuscitation (ABCs) is critical because
patients with multiple injuries or severe brain injuries may require assistance for critical functions and are unable to protect their airway. Hypoxia is
associated with increased mortality in patients with TBI, and therefore, early endotracheal intubation to secure an airway and to maintain ventilation
may be critical. Adequate analgesia and sedation are necessary for many trauma patients at intubation and early resuscitation, but the goal should be
to use the minimum amount necessary, as the loss of the neurologic examination may delay and impair critical management decisions. Maintenance of
blood pressure is also critical because hypotension (systolic blood pressure < 90 mm Hg) has been associated with a twofold increase in mortality in
TBI patients. A coagulation profile (complete blood count, prothrombin time, and partial thromboplastin time) is also recommended on admission.
Any coagulopathy, including the use of anticoagulant medications, is important to note because patients may develop sudden deterioration if there is
an underlying hemorrhage. Aggressive correction of the coagulopathy is prudent for any patients with evidence of bleeding.
The Glasgow Coma Scale
associated with increased mortality in patients with TBI, and therefore, early endotracheal intubation to secure an airway and to maintain ventilation
may be critical. Adequate analgesia and sedation are necessary for many trauma patients at intubation and early resuscitation, but the goal should be
to use the minimum amount necessary, as the loss of the neurologic examination may delay and impair critical management decisions. Maintenance of
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blood pressure is also critical because hypotension (systolic blood pressure < 90 mm Hg) has been associated with a twofold increase in mortality in
TBI patients. A coagulation profile (complete blood count, prothrombin time, and partial thromboplastin time) is also recommended on admission.
Any coagulopathy, including the use of anticoagulant medications, is important to note because patients may develop sudden deterioration if there is
an underlying hemorrhage. Aggressive correction of the coagulopathy is prudent for any patients with evidence of bleeding.
The Glasgow Coma Scale
Once initial resuscitation has been established, a primary neurologic survey should be performed to assess the severity of brain injury. The GCS is the
most widely used scoring system to evaluate the severity of the injury (see Table 38–2). The three elements of the scoring system are composed of eye
opening, verbal communication, and motor function. Patients can be classified as follows:
GCS ≤ 8 = severe
GCS 9-13 = moderate
GCS 14-15 = mild
Intubated patients are given a score of T after the number (eg, a GCS of 3T indicates the patient is intubated). It is critical that the assessment is not
clouded by sedatives or other confounding factors.
In addition to the GCS, the primary neurologic survey should also include an assessment of pupils, gross motor function, and brainstem reflexes (Table
38–3). The mass of a hematoma may cause uncal herniation, resulting in contralateral hemiparesis and unilateral pupillary dilation, requiring
emergent evacuation, and may be suspected by the examination. Potential presence of concomitant spinal cord injury should also be assessed.
Table 38–3.
Common brainstem reflexes evaluated in patients with traumatic brain injury.
Brainstem
Reflex Afferent Efferent How Tested
Level
Pupillary Cranial Midbrain CN III Light shined in the eye, observed for pupillary constriction.
nerve
(CN) II
Corneal CN V Pontine CN VII Saline dropped into the eye, observe for blink.
Vestibulo- CN VIII CN III Hold the eyes open and rotate the head side to side or up and down. Reflex present (ie,
ocular CN IV normal) if eyes move in opposite directions of head movements. Reflex not normally present
(oculocephalic) CN VI in awake patients.
Gag CN IX Medulla CN XI Pharynx stimulated with endotracheal tube or probe; observe for swallow/cough or aversion.
Diagnostic Imaging
Rapid noncontrast head CT is a critical component of all patients with moderate to severe TBI to assess for the extent of brain and skull injury.
Importantly, the head CT is reviewed for the presence of intracranial hemorrhage, which may require urgent surgical evacuation. In addition, there are
radiographic markers that predict increased ICP (Figure 38–7), which include decrease in basal and convexity subarachnoid spaces (cisternal
effacement), compression of adjacent brain structures (local mass effect), and shifting of brain contents from one hemisphere to the other (midline
shift). For patients with facial, skull base, or cervical fractures at risk for intracranial vascular injury, dedicated vascular imaging may be additionally
acquired. CTA is a convenient and rapid test commonly used as an initial detection for vascular injury, although conventional angiography remains the
gold standard. MRI is rarely used on initial neurologic evaluation but may be useful to evaluate brain tissue for more accurate prognostication of injury
at a later time.
Figure 38–7.
A : Normal head computed tomography (CT) demonstrating normal basal cisterns (white arrows). B : A head CT demonstrating effacement of basal
cisterns (black arrow) and global edema after head trauma.
effacement), compression of adjacent brain structures (local mass effect), and shifting of brain contents from one hemisphere to the other (midline
shift). For patients with facial, skull base, or cervical fractures at risk for intracranial vascular injury, dedicated vascular imaging may be additionally
acquired. CTA is a convenient and rapid test commonly used as an initial detection for vascular injury, although conventional angiography remains the
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gold standard. MRI is rarely used on initial neurologic evaluation but may be useful to evaluate brain tissue for more accurate prognostication of injury
at a later time.
Figure 38–7.
A : Normal head computed tomography (CT) demonstrating normal basal cisterns (white arrows). B : A head CT demonstrating effacement of basal
cisterns (black arrow) and global edema after head trauma.
TYPES AND MANAGEMENT OF PRIMARY TRAUMATIC BRAIN INJURY
Skull Fractures
Skull fractures can be categorized based on their mechanism, extent of injury, and location. Open skull fracture is defined as a fracture that is visible or
exposed to air and usually requires surgical management. Closed fracture may be managed surgically or medically depending on its extent of
involvement. Depressed fractures can tear the dura or lacerate the cortex of the brain, and those greater than the width of the bone are usually
considered for operative repair. Basilar skull fractures, or fractures that involve the base of the brain, are generally managed conservatively, although
one should be prudent to look for damages to the vasculature and cranial nerves and CSF leak. Clinically, one may suspect a basilar skull fracture if a
patient presents with the Battle sign, or “racoon eyes,” or hemorrhage from the ear.
Penetrating Injury & Gunshot Wounds
A penetrating injury occurs when an object, such as a bullet, causes a focal injury and penetrates the skull and the brain. Multiple factors determine the
extent of damage and outcome of the patient, including the velocity of the penetrating injury, the location, and the extent of injury to the brain. An
emergent craniotomy for removal of debris and debridement of surrounding tissue may be performed. The presence of contaminated foreign objects
increases the risk for local wound infections, meningitis, ventriculitis, and the development of cerebral abscess. The use of broad-spectrum antibiotics
is recommended in these cases, although there is variation in practices on the coverage and duration of antibiotic regimens. Head traumas due to
gunshot wounds are fatal up to 90% of the time, and for those who survive the initial trauma, half die in the emergency room. Gunshot wounds that
cross deep midline structures, the brainstem, or bilateral hemispheres are often fatal.
Intracranial Hematomas
Intracranial hematomas are collections of blood in the brain or between layers of the coverings of the brain usually due to traumatic rupture of blood
vessels. The classification of intracranial hematomas depends on the location of the hemorrhage, namely epidural, subdural, intraparenchymal, and
subarachnoid.
A. Epidural Hematoma
Epidural hematoma is bleeding that occurs outside of the dura, collecting in the space between the bone and dura. This type of hemorrhage usually
occurs from a skull fracture that lacerates a dural artery, classically the middle meningeal artery by the temporal bone, but not infrequently, also
involves laceration of a dural venous sinus. The classic presentation of a lucid interval between the initial injury and subsequent neurologic
deterioration is only present in 25% of patients with EDH. Some patients suffer no loss of consciousness on initial insult, whereas others decline
acutely after injury without a lucid interval. Radiographically, EDH appears as a lens-shaped (lentiform) hyperdense region on CT and is limited by the
neighboring dura (Figure 38–8). In general, EDHs larger than 1 cm in depth or rapidly growing hematomas on repeat imaging are considered for
subarachnoid.
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A. Epidural Hematoma
Epidural hematoma is bleeding that occurs outside of the dura, collecting in the space between the bone and dura. This type of hemorrhage usually
occurs from a skull fracture that lacerates a dural artery, classically the middle meningeal artery by the temporal bone, but not infrequently, also
involves laceration of a dural venous sinus. The classic presentation of a lucid interval between the initial injury and subsequent neurologic
deterioration is only present in 25% of patients with EDH. Some patients suffer no loss of consciousness on initial insult, whereas others decline
acutely after injury without a lucid interval. Radiographically, EDH appears as a lens-shaped (lentiform) hyperdense region on CT and is limited by the
neighboring dura (Figure 38–8). In general, EDHs larger than 1 cm in depth or rapidly growing hematomas on repeat imaging are considered for
emergent surgical removal. The operative approach involves a standard craniotomy over the EDH to allow for hematoma evacuation and to inspect for
any active dural vessels that may be coagulated. The outcome for patients with a large EDH can be quite good if managed promptly since the primary
injury does not involve the brain.
Figure 38–8.
Acute epidural hematoma after head trauma in a 5-year-old patient.
B. Acute Subdural Hematoma
Acute subdural hematoma (SDH) is bleeding that occurs under the inner surface of the dura over the surface of the brain, usually as a result of
traumatic rupture of bridging veins. This type of hemorrhage is associated with significant force, usually a diffuse, rotational force, that shears the
blood vessels. Compared to an EDH, an SDH has a more crescent shape on head CT and can extend to the entire hemisphere of the brain (Figure 38–9).
Associated intraparenchymal hemorrhage, contusion, and edema, may also be found beneath the SDH. Patients usually present with progressively
worsening mental status, and emergent operative evacuation of the hemorrhage is critical. Typically, SDHs larger than 1 cm in thickness, causing more
than 0.5 cm midline shift, or with any accompanying neurologic deficit are candidates for surgical evacuation. The operative approach involves a large
craniotomy to evacuate the hematoma. In large acute SDH, a craniectomy (with the temporary removal of bone) may be needed to allow for underlying
brain swelling. In addition, these patients should be watched closely for postoperative elevated ICP due to underlying brain contusion and edema.
Outcomes for acute SDH are often poor, with mortality of 50%-90% and with significant neurologic deficits.
Figure 38–9.
Acute subdural hematoma with an associated intraparenchymal hemorrhage after head trauma in a 25-year-old patient.
Outcomes for acute SDH are often poor, with mortality of 50%-90% and with significant neurologic deficits.
Figure 38–9.
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Acute subdural hematoma with an associated intraparenchymal hemorrhage after head trauma in a 25-year-old patient.
SDH is more prevalent in the elderly population due to atrophy of the brain, and as a result, much less force is required to stretch and tear the bridging
veins. SDH in the elderly often tends to present more chronically, even several months after injury. The timing of the hemorrhage can be discerned on
head CT, where the density of the hemorrhage is suggestive of its chronicity (Figure 38–10). The differentiation between an acute and a chronic SDH is
important because the management is different in the two scenarios. Due to decreased viscosity of old blood, surgery in chronic SDH generally
involves a much smaller craniotomy, such as a mini-craniotomy or a burr hole, to drain the old fluid. Outcomes are better than acute SDH, although
recurrence and reoperation are common.
Figure 38–10.
Chronic subdural hematoma with acute blood layering in fluid in a 55-year-old patient.
recurrence and reoperation are common.
Figure 38–10.
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Chronic subdural hematoma with acute blood layering in fluid in a 55-year-old patient.
C. Intraparenchymal Hematoma
Intraparenchymal hematoma is bleeding mixed within the brain and is the result of a direct blow to the head or at the region opposite of the point of
impact (contrecoup injury). Management for these lesions is generally conservative, although large hemorrhage (> 25 mL) may be considered for
evacuation. The relative eloquence of surrounding brain structures is taken into consideration for surgery in these cases. Patients with large
intraparenchymal hematoma are at high risk of surrounding brain edema and contusion, and close management of ICP is critical.
D. Subarachnoid Hematoma
Trauma is the most common overall cause for a subarachnoid hematoma (or subarachnoid hemorrhage) (SAH), which is blood collection in the
subarachnoid space over the convexity of the brain or in the basal cisterns. Traumatic SAH is typically associated with blood collection in the sulci or
near the surface of the brain, but blood in the basal cisterns may also be found. If there is a large density of blood collection in the basal cisterns, one
should be suspicious for a ruptured aneurysm and a subsequent angiographic study should be obtained to rule out an alternative cause of the
hemorrhage. Traumatic SAH is managed nonoperatively.
Concussion
Concussion is an immediate and transient loss of consciousness after head trauma and is often associated with amnesia. It has been thought that
concussion is a result of the disruption of the reticular activating system from the cerebral cortex rotating around the more fixed midbrain and
diencephalon.
Concussion is common, and the decision to pursue further workup, such as a CT scan, is important. In general, patients with a GCS < 15 or emesis or
who are older than age 60-65 years warrant a head CT. Patients with severe headache, intoxication, persistent anterograde amnesia, seizure, or severe
mechanism of injury should also be considered for workup.
Management of concussion includes observation for at least several hours, assuming the individual is neurologically intact. Any patients with
abnormal CT findings should be closely monitored. Long-term outcome is generally good for the majority of patients, but it is common for patients to
experience transient increased incidence of headache, memory problems, and problems with executive function even after several months.
Diffuse Axonal Injury
Diffuse axonal injury (DAI) results from rotational forces or rapid acceleration or deceleration and the traumatic shearing of axons between gray and
Concussion is common, and the decision to pursue further workup, such as a CT scan, is important. In general, patients with a GCS < 15 or emesis or
who are older than age 60-65 years warrant a head CT. Patients with severe headache, intoxication, persistent anterograde amnesia, seizure, or severe
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mechanism of injury should also be considered for workup.
Management of concussion includes observation for at least several hours, assuming the individual is neurologically intact. Any patients with
abnormal CT findings should be closely monitored. Long-term outcome is generally good for the majority of patients, but it is common for patients to
experience transient increased incidence of headache, memory problems, and problems with executive function even after several months.
Diffuse Axonal Injury
Diffuse axonal injury (DAI) results from rotational forces or rapid acceleration or deceleration and the traumatic shearing of axons between gray and
white matter. DAI is common and occurs in half of patients with severe TBI. Clinically, DAI can present in varying degrees of severity, from a mild
concussive state of confusion to coma. Head CT may demonstrate punctate (< 1 cm) hemorrhages scattered throughout the brain, but this is difficult to
visualize with this modality. MRI is more useful for detecting injury resulting from DAI for prognostication. Management of DAI is nonoperative and
primarily focused on the prevention of secondary brain injury.
TYPES AND MANAGEMENT OF SECONDARY TRAUMATIC BRAIN INJURY
Maximal medical management is directed at detecting and preventing secondary brain injuries, including increased ICP, cerebral ischemia and
hypoxia, and cerebral edema.
Physiology of Increased Intracranial Pressure
The Monro-Kellie doctrine states that intracranial volume, which is the sum of the brain, CSF, and blood, is constant. An increase in one
compartment would be accommodated by a decrease in the amount of another to maintain a constant ICP. When a mass, such as a hematoma, is
introduced into the cranial vault, CSF and venous blood are displaced to make room for the mass with relatively normal ICP. However, if the mass is
large enough to overcome these mechanisms, then ICP would rise exponentially. This simplistic model is useful for understanding increased ICP after
TBI. As secondary injury evolves with increased cerebral edema, ICP increases and results in brain compression, ischemia from decreased perfusion,
and herniation. Increased ICP is a strong predictor for mortality and morbidity after TBI.
The concept of CPP explains how increased ICP leads to decreased tissue perfusion, where CPP is defined as:
CPP = MAP – ICP

As demonstrated by this equation, an increased ICP results in a decreased CPP, leading to tissue ischemia and damage. Under normal circumstances,
MAP is between 60 and 160 mm Hg, and ICP ranges from 5 to 15 mm Hg.
This effect is further worsened by cerebral dysregulation in patients with severe TBI. Cerebral autoregulation is the mechanism that maintains CBF by
controlling cerebral vascular resistance in response to changing arterial blood pressure. For example, in a normal individual, a lowered blood pressure
leads to a decreased CPP, which would result in vasodilation to maintain a constant CBF. However, patients with severe TBI often have disruption to
autoregulation. In our example, with a decreased blood pressure and CPP, the patient has no compensatory mechanism for dilation of blood vessels,
resulting in decreased perfusion. CPP less than 70 mm Hg and hypotension are associated with increased morbidity and mortality. The intricacies of
cerebral pressure autoregulation are beyond the scope of this chapter, but a basic understanding of how increased ICP affects cerebral perfusion is
important to understand.
The incidence of raised ICP is found in 50%-60% of patients with severe TBI. Radiographic findings that should raise concerns for increased ICP include
the presence of mass effect, midline shift, the loss of sulci and gyri from the displacement of CSF, and the effacement of basal CSF cisterns. However,
increased ICP can still occur without any radiographic evidence and should be suspected in patients with severe TBI or with a low GCS score.
ICP can be directly measured by the insertion of a monitoring device into the brain, typically performed by the bedside in the emergency room or the
ICU. The device can be placed in the epidural, subdural, intraparenchymal, or intraventricular space. The most common devices currently used are the
intraparenchymal ICP monitor and the intraventricular catheter, also more commonly known as the external ventricular drain (EVD). The
intraparenchymal monitor is a device that places the sensor directly into brain tissue to monitor ICP. This monitor has a lower rate of intracranial
hemorrhage and infection compared with the EVD but is subject to drift in accuracy by 3-4 mm Hg after 4-5 days.
The EVD is a commonly performed procedure that involves the placement of a catheter through the brain parenchyma into the lateral ventricles and
serves as both a diagnostic and therapeutic device. The EVD is more invasive but allows for CSF drainage as a therapeutic benefit to decrease ICP.
Management of Increased Intracranial Hypertension

A. Optimization of Cerebral Venous Outflow
Elevation of the head of the bed to 15-30 degrees is the standard of care to reduce ICP by minimizing venous outflow resistance. In patients with TBI
who may also have concurrent or unknown spinal injuries, a tight-fitting cervical collar can increase ICP. Loosening the collar, if deemed safe, may
hemorrhage and infection compared with the EVD but is subject to drift in accuracy by 3-4 mm Hg after 4-5 days.
The EVD is a commonly performed procedure that involves the placement of a catheter through the brain parenchyma into the lateral ventricles and
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serves as both a diagnostic and therapeutic device. The EVD is more invasive but allows for CSF drainage as a therapeutic benefit to decrease ICP.
Management of Increased Intracranial Hypertension
A. Optimization of Cerebral Venous Outflow
Elevation of the head of the bed to 15-30 degrees is the standard of care to reduce ICP by minimizing venous outflow resistance. In patients with TBI
who may also have concurrent or unknown spinal injuries, a tight-fitting cervical collar can increase ICP. Loosening the collar, if deemed safe, may
improve ICP.
B. CSF Drainage
Currently, according to the guidelines from the Brain Trauma Foundations, it remains uncertain whether the use of EVD reduces mortality, but a Level
III recommendation suggests the use of CSF drainage to lower ICP in patients with GCS < 6 during the first 12 hours after injury. Threshold for
treatment after TBI is typically > 20 mm Hg in adult patients.
C. Seizure Prevention
The incidence of seizures after a TBI is estimated to be 5%-15%, with the majority occurring within the first 7 days after trauma. Seizure increases
cerebral metabolic rate and ICP and may be subclinical in patients with severe TBI. Prophylactic anticonvulsants, such as phenytoin or levetiracetam,
are commonly used to prevent seizures after a moderate to severe TBI during the first week after trauma. Prophylactic use of antiepileptics is not
recommended for preventing late seizures or the development of epilepsy.
D. Hyperosmolar Therapy
Mannitol is a commonly used hyperosmolar agent for the reduction of increased ICP. Doses of 0.25-1 g/kg body weight are typically used and usually
lower ICP in 1-5 minutes, with peak effects at 20-60 minutes. Arterial hypotension (systolic blood pressure < 90 mm Hg) should be avoided. Mannitol
can be redosed every 6 hours for long-term ICP management, although careful attention should be paid to the occurrence of mannitol-induced
diuresis and renal impairment. Mannitol has both rheologic and osmotic effects by increasing CBF through the expansion of plasma volume and by
acting as a free radical scavenger.
Hypertonic saline, given usually in concentrations of 3% or 23.4%, creates an osmotic force to draw water from interstitial space of the brain
parenchyma into the intravascular compartment to reduce ICP. Hypertonic saline is preferred over mannitol in hypovolemic and hypotensive patients
because it augments intravascular volume and increases blood pressure. Hyponatremia should be excluded before administering hypertonic saline to
reduce the risk of central pontine myelinolysis.
E. Hyperventilation
Hyperventilation decreases PaCO2, leading to vasoconstriction and decreases in cerebral blood volume and ICP. Although hyperventilation was once
practiced widely, it has now been regarded to play a limited role in the management of intracranial hypertension, as persistent hyperventilation may
lead to ischemia. Hyperventilation is recommended as a temporizing measure for reduction of ICP, but prolonged use is contraindicated.
F. Sedation
Pain and agitation can significantly increase ICP, and therefore, the use of adequate sedation and analgesia is an important element of ICP
management. The use of sedation with or without chemical paralysis also aids in reducing excessive brain metabolism that accompanies severe TBI.
Shorter-acting agents are preferred to allow for frequent assessment of neurologic examination.
G. Barbiturate Coma
The use of high-dose barbiturates is generally reserved for refractory intracranial hypertension. The exact mechanism by which barbiturates lower ICP
is unclear, but it is likely associated with reduction of cerebral metabolism by the coupled reduction in CBF and cerebral metabolic rate of oxygen.
However, there are serious complications associated with this treatment, including severe hypotension, metabolic derangements, respiratory
complications, infections, and hepatic and renal dysfunction.
H. Fever and Hypothermia
Fever is associated with increased metabolic rate and induces dilation of cerebral vessels, which may increase ICP. Fever has been associated
Chapter 38: Neurosurgery, Timothy R. Smith Pagewith
29 / 93
worse neurologic outcome and should be controlled with antipyretics and cooling blankets.
Hypothermia has been shown to slow edema and injury at a cellular level in experimental settings, and although fever has been shown to be associated
is unclear, but it is likely associated with reduction of cerebral metabolism by the coupled reduction in CBF and cerebral metabolic rate of oxygen.
However, there are serious complications associated with this treatment, including severe hypotension, metabolic derangements, respiratory
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complications, infections, and hepatic and renal dysfunction.
H. Fever and Hypothermia
Fever is associated with increased metabolic rate and induces dilation of cerebral vessels, which may increase ICP. Fever has been associated with
worse neurologic outcome and should be controlled with antipyretics and cooling blankets.
Hypothermia has been shown to slow edema and injury at a cellular level in experimental settings, and although fever has been shown to be associated
with worse outcome, randomized clinical trials of prophylactic hypothermia have failed to show benefit. Current guidelines do not recommend
prophylactic hypothermia for the management of elevated intracranial hypertension.
I. Steroids
Large randomized trials of methylprednisolone in severe TBI patients were associated with increased mortality. The use of steroids is not
recommended for management of ICP.
J. Surgical Decompressive Craniectomy
Decompressive craniectomy is the maximal aggressive measure for management of increased ICP and involves removal of a portion of the calvarium to
allow for increased space. The literature has found surgical decompression to be associated with decreased ICP, but its effect on overall neurologic
outcome is debated. In clinical practice, decompressive craniectomy is considered the last resort for when maximal medical treatment has failed to
control ICP.
CLINICAL OUTCOMES AFTER TRAUMATIC BRAIN INJURY
The medical literature is mixed on long term clinical outcome after TBI, but it has been reported that up to one-third of patients with severe TBI
succumb to death or are severely disabled at 6 months. In those who survive, over 50% have identifiable disability 1 year after injury. Predictors for
poor outcome include older age, worse initial GCS score, hypotension, hypoxia, elevated ICP, extent of intracranial injury, and existing comorbidities.
Unfortunately, for some patients with a severe degree of injury, the likelihood of a meaningful recovery may be futile. Once the diagnosis and injury
severity are confirmed, with unchanged neurologic condition despite maximal resuscitation, a frank discussion with the family is necessary to make
appropriate decisions on the degree of aggressive care as per the patient’s and family’s wishes.
SUMMARY
TBI is a devastating condition with a high burden on young individuals; it also poses a major societal burden. For patients with severe TBI, initial
resuscitation, immediate recognition and diagnosis, emergent surgery if needed, and management of subsequent secondary injuries are critical for
optimal outcome. Trauma patients with severe TBI may need to be transferred to an institution well equipped to handle major neurologic injuries after
initial assessment and stabilization.
Carney N, et al: Guidelines for the Management of Severe Traumatic Brain Injury, Fourth Edition. Neurosurgery . 2017;80(1):6–15. [PubMed: 27654000]
Rangel-Castillo L, et al: Management of intracranial hypertension. Neurol Clin . 2008;26(2):521–541. [PubMed: 18514825]
SPINAL CORD INJURY

Ian Tafel, MD, and Timothy R. Smith, MD, PhD
General Considerations
Traumatic spinal cord injury (SCI) primarily affects young people, with a median age at diagnosis of 37.6 years, and often results in significant disability
or death. The median age of SCI has increased from 28.7 years in the 1970s largely due to an increase in the incidence of falls causing SCI in patients
older than 60 years of age. The main causes in order of incidence are motor vehicle collision, fall, violence, and sports injuries. The prognosis for
significant recovery of a complete lesion is poor. Recent research into stem cell technology and other new modalities of therapy is, however, showing
promise.
The cost of SCI to the healthcare system and society is significant. A 25-year-old patient with a high cervical cord injury (C1-4) is estimated to incur
$741,425 in medical costs in the first year following injury and $132,807 for each year survived thereafter. In addition, the loss of wages and productivity
for SCI patients averages $57,000 annually. With 12,000-14,000 Americans suffering SCI per year, the social and economic costs are significant.
Traumatic spinal cord injury (SCI) primarily affects young people, with a median age at diagnosis of 37.6 years, and oftenInternational Medical University
results in significant disability
or death. The median age of SCI has increased from 28.7 years in the 1970s largely due to an increase in the incidence ofAccess Provided by:
falls causing SCI in patients
older than 60 years of age. The main causes in order of incidence are motor vehicle collision, fall, violence, and sports injuries. The prognosis for
significant recovery of a complete lesion is poor. Recent research into stem cell technology and other new modalities of therapy is, however, showing
promise.
The cost of SCI to the healthcare system and society is significant. A 25-year-old patient with a high cervical cord injury (C1-4) is estimated to incur
$741,425 in medical costs in the first year following injury and $132,807 for each year survived thereafter. In addition, the loss of wages and productivity
for SCI patients averages $57,000 annually. With 12,000-14,000 Americans suffering SCI per year, the social and economic costs are significant.
Treatment for SCI consists of acute and chronic management. Acutely, the ABCs (airway, breathing, and circulation) must be secured and the spine
immobilized to prevent extension of injuries. Compressive lesions must be identified and the need for urgent surgical management determined.
Although there is debate as to whether the administration of methylprednisolone after SCI significantly improves motor score, the American
Association of Neurological Surgeons/Congress of Neurological Surgeons (AANS/CNS) does not recommend its administration. Other acute
nonsurgical treatment options aimed at minimizing secondary injury are modest induced hypothermia and hyperbaric therapy; the former has not
been proven to have beneficial effects in humans, whereas the latter increases the speed of neurologic recovery, but does not result in an overall
improvement in outcome. Chronic management includes physical and occupational therapy designed to maximize functionality. Specific therapy and
improvement depend on the level and completeness of the injury.
Clinical Findings
Clinical findings in SCI depend on the level, mechanism, and severity of injury, with injuries being classified as either complete or incomplete. A
complete SCI refers to the lack of motor or sensory function below the level of the lesion, while incomplete lesions have some preserved function.
Incomplete lesions often result in recognized SCI syndromes based on the region of the spinal cord affected. The American Spinal Injury Association
(ASIA) publishes the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) scale to further classify the severity of SCI
(Table 38–4).
Table 38–4.
The ASIA classification of spinal cord injury.
A = Complete: No sensory or motor function preserved in the lowest sacral segments (S 4/5).
B = Sensory incomplete: Sensory but no motor function preserved below the neurologic level including the sacral segments S4/5.
C = Motor incomplete: Motor function is preserved below the neurologic level, and more than half of the key muscles below the neurologic level have a
muscle grade less than 3. There must be some sparing of sensory and/or motor function in the segments S4/5.
D = Motor incomplete: Motor function is preserved below the neurologic level, and more than half the key muscles below the neurologic level have a
muscle grade greater than or equal to 3. There must be some sparing of sensory and/or motor function in the segments S4/5.
E = Normal: Sensory and motor functions are normal. Patient may have abnormalities on reflex examination.
Adapted with permission from American Spinal Injury Association: Standards for Neurological Classification of Spinal Cord Injury (rev. 2000). Chicago: ASIA, 2002.
Initial clinical findings include motor or sensory deficit and hyporeflexia. Initially, all reflexes below the lesion are lost, including the bulbocavernosus,
cremasteric, and abdominal cutaneous reflexes, depending on the level of injury. Over time, these reflexes may return, and the deep tendon reflexes
may become hyperreflexive due to the loss of descending tonic inhibition of the reflex arc. Initially, paralysis is flaccid, but eventually, upper motor
neuron signs develop, and a spastic paralysis results. If the lesion is in the high cervical region (C1-5), respiratory effort may be compromised due to
the loss of innervation to the phrenic nerve. Loss of bowel or bladder function often occurs, and loss of rectal tone and sensation, as well as priapism,
may result. The loss of bladder control manifests as urinary retention, and developing urinary incontinence is typically overflow incontinence. Loss of
anal sphincter tone and sensation results in leakage of stool and lack of awareness of bowel movements.
An important early finding that can occur in SCI is “spinal shock.” This refers to a drop in the systolic blood pressure with accompanying bradycardia,
often to a level of 80 mm Hg systolic, following SCI. This is due to the loss of sympathetic tone to the regions below the lesion and causes venous
pooling and decreased venous return to the heart.
Chronic clinical findings in SCI are related to the long-term need for ventilatory support, immobilization, and need for catheterization. Pneumonia,
urinary tract infections, and decubitus ulcers are common findings and are often the cause of death in spinal cord–injured patients.
Incomplete SCIs may demonstrate a variable pattern of sensory or motor preservation, although they can often be categorized into recognizable
clinical syndromes, depending on the mechanism of injury and the portion of the cord affected. Page 31 / 93
A. Central Cord Syndrome
often to a level of 80 mm Hg systolic, following SCI. This is due to the loss of sympathetic tone to the regions below the lesion and causes venous
pooling and decreased venous return to the heart. International Medical University
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Chronic clinical findings in SCI are related to the long-term need for ventilatory support, immobilization, and need for catheterization. Pneumonia,
urinary tract infections, and decubitus ulcers are common findings and are often the cause of death in spinal cord–injured patients.
Incomplete SCIs may demonstrate a variable pattern of sensory or motor preservation, although they can often be categorized into recognizable
clinical syndromes, depending on the mechanism of injury and the portion of the cord affected.
A. Central Cord Syndrome
Central cord syndrome refers to a pattern of injury that affects the motor strength in the upper extremities more severely than the lower extremities.
Sensory function is variable below the level of the lesion, and sphincter control is often affected. This usually occurs in older patients with spinal
stenosis following a hyperextension injury. The central cervical cord is a watershed vascular territory that is thought to be disrupted in this syndrome.
The spinal cord is somatotopically organized such that cervical fibers are more medial compared to fibers traveling to the lower extremities, resulting
in more severely affected upper extremities.
B. Anterior Cord Syndrome
Anterior cord syndrome results from the compression of the anterior portion of the cord by a herniated disk or bone fragment or from occlusion of the
anterior spinal artery. The corticospinal tracts and spinothalamic tracts are preferentially affected due to their more anterior location, whereas the
posterior columns are relatively spared. This results in loss of motor function and loss of pain and temperature sensation below the level of the lesion,
with preserved proprioception, vibration, and pressure sensation. It is important to distinguish surgical from nonsurgical (ie, anterior spinal artery
occlusion) etiologies in this condition.
C. Brown-Séquard Syndrome
Brown-Séquard syndrome occurs after spinal cord hemisection. It is usually the result of penetrating trauma and occurs in 2%-4% of SCIs. Motor and
posterior column function (proprioception, vibration sense) are disrupted on the side of the lesion. Pain and temperature sensation are diminished on
the contralateral side due to the crossing of the spinothalamic tract in the spinal cord at one to two levels above the entrance of the fibers into the cord.
D. Conus Medullaris Syndrome
Conus medullaris syndrome results from injury to the sacral spinal cord. Symptoms include saddle anesthesia, loss of bowel/bladder function, and
lower extremity weakness. It includes a combination of both upper and lower motor neuron signs.
E. Cauda-Equina Syndrome
Cauda-equina syndrome refers to compression and dysfunction of the lumbosacral nerve roots. It is not a true SCI because it only affects the nerve
roots and not the cord itself. The clinical syndrome is similar to conus medullaris syndrome with saddle anesthesia, loss of bowel/bladder function,
and lower extremity weakness, but the findings are all lower motor neuron.
Physical Examination
Initial physical examination in SCI focuses on the ABCs (airway, breathing, and circulation). Special attention must be focused on the airway in high
cervical injuries because patients may require endotracheal intubation. Blood pressure must be closely monitored given the possibility of spinal shock.
This manifests as a drop in the systolic blood pressure and must be addressed immediately to prevent further cord ischemia.
In the awake patient, a history focusing on the mechanism of injury and detailed neurologic examination is undertaken to determine the level and
completeness of the injury. Motor strength should be tested in all muscle groups and sensation should be tested with pinprick, and proprioception.
Rectal tone and sensation should be tested with digital examination. Reflexes should be examined, including the bulbocavernosus, cremasteric, and
abdominal cutaneous reflexes. Careful palpation of the spine is important to evaluate for obvious step-offs or tenderness to palpation at all levels. The
examination must be carefully documented and a neurologic level and evaluation of completeness of the lesion determined.
In the comatose patient, a complete neurologic examination is often difficult. In this situation, observation of spontaneous movements or movements
to painful stimuli is important. Deep tendon reflexes should be examined, and palpation of the spine should be undertaken to observe for obvious
step-offs. Radiologic imaging is often required to adequately determine a level of injury and its etiology.
Differential Diagnosis
History, physical examination, and radiographic imaging generally arrive at the diagnosis of SCI. Moreover, radiographic imaging can helpPage
Chapter 38: Neurosurgery, Timothy R. Smith 32 / 93
determine
the mechanism of the injury. Some peripheral nerve lesions may resemble SCI, but these are typically unilateral and affect only lower motor neurons.
Malingering and conversion disorders may mimic SCI and should be suspected in the setting of serial examinations showing inconsistencies, as well as
imaging findings that do not explain the symptoms.
examination must be carefully documented and a neurologic level and evaluation of completeness of the lesion determined.
In the comatose patient, a complete neurologic examination is often difficult. In this situation, observation of spontaneous movements or movements
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to painful stimuli is important. Deep tendon reflexes should be examined, and palpation of the spine should be undertaken to observe for obvious
step-offs. Radiologic imaging is often required to adequately determine a level of injury and its etiology.
History, physical examination, and radiographic imaging generally arrive at the diagnosis of SCI. Moreover, radiographic imaging can help determine
the mechanism of the injury. Some peripheral nerve lesions may resemble SCI, but these are typically unilateral and affect only lower motor neurons.
Malingering and conversion disorders may mimic SCI and should be suspected in the setting of serial examinations showing inconsistencies, as well as
imaging findings that do not explain the symptoms.
Radiologic Examination
According to the current recommendations of the AANS/CNS on cervical spine injury, in the awake, asymptomatic patient with no spinal tenderness,
distracting injury (eg, long-bone fracture), or evidence of disturbed consciousness or intoxication, no radiographic imaging is necessary and no
cervical immobilization is recommended. However, awake patients with spine tenderness, numbness, tingling, or obvious signs of SCI (eg, weakness,
loss of bowel/bladder control) require a CT scan, or if one is not available, three-view cervical spine x-rays should be obtained (Figure 38–11). In the
awake, symptomatic, patient with a normal CT, an MRI should be obtained within 48 hours of injury to rule out ligamentous injury. If the MRI or
flexion/extension x-rays are not concerning for injury, then the cervical immobilization may be removed. Additionally, MRI (particularly T2-weighted
sequences) also clearly demonstrates compression of and/or signal change within the spinal cord (Figure 38–12).
Figure 38–11.
Sagittal reconstruction of a cervical computed tomography (CT) scan demonstrating a C6-7 traumatic fracture.
sequences) also clearly demonstrates compression of and/or signal change within the spinal cord (Figure 38–12).
Figure 38–11.
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Sagittal reconstruction of a cervical computed tomography (CT) scan demonstrating a C6-7 traumatic fracture.
Figure 38–12.
Sagittal T2-weighted magnetic resonance image of a C6-7 traumatic fracture demonstrating spinal cord compression and signal change within the
spinal cord (arrow).
Figure 38–12.
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Sagittal T2-weighted magnetic resonance image of a C6-7 traumatic fracture demonstrating spinal cord compression and signal change within the
spinal cord (arrow).
Obtunded or unevaluable patients with suspected cervical spine injury require CT or three-view cervical spine x-ray if CT is not available and should
have an MRI within 48 hours of injury if no injury is noted on the CT or x-ray. Clearance of the cervical spine in this population is still cause for debate,
and recommendations are in flux. Generally, however, a lack of injury noted on CT (or x-ray) and MRI allows for discontinuation of cervical spine
immobilization at the discretion of the treating physician.
Treatment
Initial treatment of SCI consists of securing the ABCs and immobilizing the spinal column. In the case of high cervical spine injury, the need for
endotracheal intubation must be identified, and if it is not immediately necessary, serial arterial blood gas assessments should be monitored to
evaluate for hypocapnia and progressive ventilatory failure. Spinal shock and the resultant decrease in blood pressure must be treated aggressively if
they occur. Volume expansion should be initiated promptly, and decreased systolic blood pressure refractory to volume expansion should be treated
with vasopressor therapy with a MAP goal of 85-90 mm Hg for 7 days following injury. The choice of pressors has not been conclusively defined, but
typically a beta-agonist is followed by an alpha-agonist given the possibility of bradycardia in spinal shock.
Patients should be placed in a hard cervical collar, and cervical immobilization should be ensured until clearance of the cervical spine or definitive
treatment has occurred. Patients should be placed on a board for transfers and log-rolled for movement until the thoracic and lumbar spines are
cleared as well. Other initial management considerations include placement of an arterial line to monitor blood pressure on a constant basis and
placement of a Foley catheter to decompress the bladder. The AANS/CNS does not recommend administration of methylprednisolone after cervical
spine injury, citing inconsistent evidence of its benefit and the risk of adverse events such as wound infection, hyperglycemia, and gastrointestinal
hemorrhage.
Page 35 / 93
Following initial stabilization and imaging studies, the need for surgical intervention is assessed. Surgery has two main goals: decompression and
stabilization. Surgery is employed on an emergent basis for incomplete lesions in the hopes of preserving or improving neurologic function and on a
typically a beta-agonist is followed by an alpha-agonist given the possibility of bradycardia in spinal shock.
Patients should be placed in a hard cervical collar, and cervical immobilization should be ensured until clearance of theAccess Provided by:
cervical spine or definitive
treatment has occurred. Patients should be placed on a board for transfers and log-rolled for movement until the thoracic and lumbar spines are
cleared as well. Other initial management considerations include placement of an arterial line to monitor blood pressure on a constant basis and
placement of a Foley catheter to decompress the bladder. The AANS/CNS does not recommend administration of methylprednisolone after cervical
spine injury, citing inconsistent evidence of its benefit and the risk of adverse events such as wound infection, hyperglycemia, and gastrointestinal
hemorrhage.
Following initial stabilization and imaging studies, the need for surgical intervention is assessed. Surgery has two main goals: decompression and
stabilization. Surgery is employed on an emergent basis for incomplete lesions in the hopes of preserving or improving neurologic function and on a
nonemergent basis for complete lesions, as there is no demonstrated improvement in neurologic function for emergent surgery in these situations.
Goals of surgery in this setting are to prevent cranial extension of injury and to prevent progressive deformity. The choice of surgical approach for SCI
is not standardized and is dependent on the location of the pathology. Current stabilization procedures typically involve instrumented fusion
techniques and may be approached via an anterior, posterior, or combined approach.
Cervical traction may be employed either alone or as an adjunct to surgical therapy to attempt realignment of the spinal column. This is accomplished
by fixing a halo ring or specialized devices (eg, Gardner-Wells tongs) to the head, connecting this to a rope and pulley system, and adding weight to
adjust the spine in the desired vector.
Chronic treatment of SCI focuses on rehabilitation and adaptation to permanent injury. Rehabilitation can often result in improved neurologic
function in incomplete lesions and can help those with complete injuries become as functional as possible. Patients may require ventilatory support,
tracheostomy, intermittent catheterization, frequent turning (to prevent decubitus ulcers), and functional accommodations such as wheelchairs and
other devices aimed at improving functionality. Attention to long-term care issues can prolong the life and productivity of SCI patients.
Prognosis & Outcome
Death in the acute trauma setting from SCI is 20%. Complete lesions that remain so at 72 hours are unlikely to improve beyond one level above the
lesion in the long term. Patients with quadriplegia who have initial ventilator dependency have 5-year survival rates of approximately 33%. Incomplete
lesions have a more favorable outcome. Among recognized SCI syndromes, central cord syndrome and Brown-Séquard syndrome have the most
favorable outcomes, with up to 90% of patients being able to ambulate independently at 1 year. Anterior cord syndrome patients have a worse
prognosis, with 10%-20% recovering functional motor control. Causes of death in long-term SCI patients are usually due to cardiac, respiratory, or
infectious causes, often related to the sequelae of SCI.
Current multidisciplinary approaches to SCI involve emergency department, medical, surgical, and rehabilitation staff providing the best possible
therapy for patients. Despite this, SCI remains a devastating injury with high rates of mortality and permanent disability. Novel research and
innovations will assuredly provide better outcomes for SCI in the future.
Badhiwala JH, Wilson JR, Kwon BK, et al: A review of clinical trials in spinal cord injury including biomarkers. J Neurotrauma . 2018;35(16):1906–1917.
[PubMed: 29888678]
Hadley MN, Walters BC: Introduction of the guidelines for the management of acute cervical spine and spinal cord injuries. Neurosurgery . 2013;72:5–
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Hurlbert RJ, Hadley MN, Walters BC, et al: Pharmacological therapy for acute spinal cord injury. Neurosurgery. 2013;72:93–105. [PubMed: 23417182]
Kirshblum S, Waring W 3rd: Updates for the international standards for neurological classification of spinal cord injury. Phys Med Rehabil Clin N Am .
2014;25:505–517. [PubMed: 25064785]
Kwon BK, Mann C, Sohn HM, et al: Hypothermia for spinal cord injury. Spine J . 2008;8(6):859–874. [PubMed: 18329959]
McKinley W, Santos K, Meade M, et al: Incidence and outcomes of spinal cord injury clinical syndromes. J Spinal Cord Med. 2007;30:215–224.
[PubMed: 17684887]
Priebe MM, Chiodo AE, Scelza WM, et al: Spinal cord injury medicine 6. Economic and societal issues in spinal cord injury. Arch Phys Med Rehabil.
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Kwon BK, Mann C, Sohn HM, et al: Hypothermia for spinal cord injury. Spine J . 2008;8(6):859–874. [PubMed: 18329959]
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McKinley W, Santos K, Meade M, et al: Incidence and outcomes of spinal cord injury clinical syndromes. J Spinal Cord Med. 2007;30:215–224.
[PubMed: 17684887]
Priebe MM, Chiodo AE, Scelza WM, et al: Spinal cord injury medicine 6. Economic and societal issues in spinal cord injury. Arch Phys Med Rehabil.
2007;88(Suppl 1):S84–88. [PubMed: 17321854]
PERIPHERAL NERVE LESIONS

Familiarity with the pertinent aspects of peripheral nerve anatomy and physiology combined with focused history and physical examination aids in the
management of peripheral nerve lesions. The history and physical examination may then be complimented by electrodiagnostic and radiographic
studies.
Anatomy
Peripheral nerves are composed of varying combinations of sensory and motor axons from more than one spinal nerve root, and each spinal nerve
root contributes fibers to more than one peripheral nerve. An axon is a long projection from a nerve cell body that is bounded by a cell membrane as
well as a basement membrane. Some axons are surrounded by sheaths of myelin, a fatty substance secreted by Schwann cells. Myelin insulates the
axon, thereby increasing the velocity of neurotransmission. The axon is, in turn, surrounded by a layer of connective tissue called endoneurium. Axons
travel together in bundles called fascicles, each of which is covered by another layer of connective tissue called perineurium. Fascicles are grouped
together to form a peripheral nerve, which is surrounded by a final layer of connective tissue called epineurium.
ACUTE PERIPHERAL NERVE INJURIES
Lesions of spinal nerves manifest as radiculopathies with blurred sensory disturbances, whereas those in peripheral nerves demonstrate sharply
demarcated sensory disturbances. Spinal nerve lesions result in mild to moderate weakness in muscles supplied by one spinal nerve but by more than
one peripheral nerve. Peripheral nerve lesions manifest more severe muscle atrophy and weakness in muscles supplied solely by the peripheral nerve.
Common etiologies of acute peripheral nerve injury include penetrating or blunt trauma, traction, fractured bones, and compression from
hematomas. Minor peripheral nerve injuries arise from blunt trauma that temporarily compresses or stretches a nerve while leaving its axons intact
(neurapraxia). In such cases, axonal transport may be temporarily impaired, but Wallerian degeneration, or the death of axons distal to the point of
injury, does not occur. These injuries generally recover spontaneously over the course of days to weeks. Slightly more severe injuries may interrupt
axons and their myelin sheaths while leaving endoneurium intact (axonotmesis). In these cases, Wallerian degeneration inevitably follows. Axonal
regeneration may occur spontaneously, however, guided to areas of previous innervation by intact endoneurial tubes. With this type of injury, there is
a good prognosis for spontaneous functional recovery, with axonal regeneration occurring at a rate of about 1 mm/d or 1 inch per month.
If the ends of a completely divided nerve (neurotmesis) remain in proximity, regeneration can occur via axonal sprouting from the proximal stump.
These axonal sprouts may bridge the gap to the distal stump, propagating through preserved endoneurial tubes at a rate of 1 mm/d. Severe crush
injuries may create internal damage without complete transection, resulting in disruption of axons and their endoneurium and disturbance of the
organization of fascicles within the nerve. In these cases, fibrous scar tissue may form within the macerated nerve, which can block the regeneration of
axonal sprouts. A tangle of axonal sprouts contained in fibrous scar tissue is called a neuroma, and these act as a barrier to spontaneous peripheral
nerve regeneration.
Clinical Findings
A detailed clinical history and neurologic examination help determine which peripheral nerves have been injured and the type of injury present. The
type of trauma will generally suggest whether the nerve is in continuity. A penetrating injury with a sharp object, such as a knife, suggests a clean
transection that is amenable to immediate surgical repair. Nonpenetrating trauma or a stretch injury is more suggestive of nerve continuity.
Determine the timing of motor and sensory deficits may also aid in the assessment of the nerve injury. For example, in the setting of a penetrating
sharp injury, an immediate deficit at the time of the injury would suggest direct involvement of the nerve. However, a delayed deficit wouldPage
Chapter 38: Neurosurgery, Timothy R. Smith 37 / an
suggest 93
enlarging adjacent lesion such as a hematoma or pseudoaneurysm.
The physical examination includes inspection, observation, evaluation of the relevant vasculature, range of motion assessment, and neurological
Clinical Findings
A detailed clinical history and neurologic examination help determine which peripheral nerves have been injured and the type of injury present. The
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type of trauma will generally suggest whether the nerve is in continuity. A penetrating injury with a sharp object, such as a knife, suggests a clean
transection that is amenable to immediate surgical repair. Nonpenetrating trauma or a stretch injury is more suggestive of nerve continuity.
Determine the timing of motor and sensory deficits may also aid in the assessment of the nerve injury. For example, in the setting of a penetrating
sharp injury, an immediate deficit at the time of the injury would suggest direct involvement of the nerve. However, a delayed deficit would suggest an
enlarging adjacent lesion such as a hematoma or pseudoaneurysm.
The physical examination includes inspection, observation, evaluation of the relevant vasculature, range of motion assessment, and neurological
examination. A laceration, fracture, or bruising/abrasions may suggest the site of an underlying nerve injury. The Horner sign (ptosis, meiosis, and
anhidrosis) is suggestive of a proximal T1/lower trunk brachial plexus lesion. An impaired range of motion can make assessment of strength difficult to
evaluate accurately. Furthermore, an elevated hemidiaphragm is suggestive of a phrenic nerve injury.
The sensory and motor findings associated with acute peripheral nerve injury vary widely, depending on which nerve is injured. Pain may also be a
symptom, but it usually develops in a delayed fashion. Pain can occur because of neuroma formation, where it is often associated with a tender lump in
the area of injury. Neurogenic pain may also develop because of a disturbance in the processing of pain signals. This type of pain, when associated
with autonomic hyperfunction, is referred to as complex regional pain syndrome (formally known as causalgia or reflex sympathetic dystrophy), and it
is notoriously difficult to treat. When neurogenic pain is associated with nerve root avulsion, it is known as deafferentation pain. Deafferentation pain
often responds well to surgical intervention via dorsal root entry zone ablation.
In the diagnosis of acute peripheral nerve injury, electromyography and nerve conduction studies are generally not useful until at least 3 weeks after
injury. Nevertheless, it is important to obtain baseline electrodiagnostic studies because they are important for monitoring recovery. In the case of
brachial plexus injury, it is useful to obtain an MRI scan or CT myelogram to look for pseudomeningoceles in the vicinity of the nerve roots, which
would indicate nerve root avulsion.
Certain peripheral nerve injuries are associated with traumatic fractures of specific bones. For example, traumatic injuries of the radial nerve
classically occur with fractures of the shaft of the humerus, at the level of the spiral groove. Such injuries result in weakness of wrist extension, finger
extension, and thumb extension, as well as numbness over the radial aspect of the dorsal surface of the hand. In this type of injury, elbow extension is
not affected since muscular branches to the triceps are given off proximal to the spiral groove.
Trauma to the brachial plexus can cause a wide array of neurologic signs and symptoms. Clinical manifestations are determined by both the location
and severity of the injury. Erb-Duchenne palsy is due to an injury primarily to the upper trunk of the brachial plexus (derived from C5 and C6 nerve
roots). It typically results from a stretch injury such as traction on the arm at the time of birth or a fall that forcefully separates the head from the
shoulder. The deltoid, biceps, rhomboids, brachioradialis, supraspinatus, and infraspinatus are affected, leaving the arm hanging to the side,
internally rotated, and extended at the elbow, which is called the “waiter’s tip position.”
In acute trauma, when unilateral limb findings are present, a thorough neurologic exam is critical to differentiate acute radiculopathy from peripheral
nerve injury. Several general principles should be considered. Radiculopathy is often accompanied by neck or back pain, which tends to radiate down
an arm or a leg. Also the sensory findings of radiculopathy tend to be blurred, reflecting the overlapping nature of dermatomes, while sensory findings
in peripheral nerve injuries are sharply demarcated. Weakness from radiculopathy occurs in muscles innervated by one spinal nerve but by more than
one peripheral nerve. Thus, it is often only partial weakness, because nearly all muscles are innervated by more than one spinal nerve.
Acute trauma to a peripheral nerve usually results in maximal deficits at the time of injury, but a deficit that progresses should initiate further workup.
Immediate surgical exploration should be considered to address compressive lesions such as expanding hematomas or growing traumatic
pseudoaneurysms. Sources of ongoing neurologic compression should be removed as soon as possible.
Treatment & Prognosis
Sharp nerve transections with clean ends should be repaired within 3 days. In transections that are less than 5 mm, the repair should be performed in
an end-to-end fashion, with no tension across the repair site. However, for larger defects, an autologous nerve graft should be used. Transections
from penetrating trauma that do not have clean edges or where significant tissue loss is present should be repaired in a delayed fashion. During
exploration of the wound, transected nerve stumps should be identified and tagged. The tagged nerve ends should be attached to fascia to reduce the
possibility of retraction. After 3 weeks, the lesion should be reexplored and the injured nerve repaired. At that time, areas of axonal damage and
neuroma formation are easier to visualize. Better visualization of damaged axons reduces the possibility of subsequent neuroma formation at the site
of repair.
In the case of nonpenetrating injury, recovery often occurs spontaneously without the need for surgery. In these cases, nonoperative management
with serial neurologic examinations, including electrophysiologic testing, should be the initial treatment modality. If, after 3 months, there is no sign of
clinical recovery, the injured nerve should be explored. Intraoperative electrophysiologic nerve mapping should be performed to determine whether
an end-to-end fashion, with no tension across the repair site. However, for larger defects, an autologous nerve graft should be used. Transections
from penetrating trauma that do not have clean edges or where significant tissue loss is present should be repaired in aInternational Medical University
delayed fashion. During
exploration of the wound, transected nerve stumps should be identified and tagged. The tagged nerve ends should be attached to fascia to reduce the
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possibility of retraction. After 3 weeks, the lesion should be reexplored and the injured nerve repaired. At that time, areas of axonal damage and
neuroma formation are easier to visualize. Better visualization of damaged axons reduces the possibility of subsequent neuroma formation at the site
of repair.
In the case of nonpenetrating injury, recovery often occurs spontaneously without the need for surgery. In these cases, nonoperative management
with serial neurologic examinations, including electrophysiologic testing, should be the initial treatment modality. If, after 3 months, there is no sign of
clinical recovery, the injured nerve should be explored. Intraoperative electrophysiologic nerve mapping should be performed to determine whether
there is conduction across the site of injury. If there is no conduction of evoked potentials, the neuroma should be resected and the stubs should be
trimmed and brought together primarily without tension. If intraoperative stimulation reveals conduction across an area of injury, then the nerve
should be left intact and allowed to regenerate on its own. A mnemonic for remembering the appropriate timing of operative repair for traumatic nerve
injuries is “the rule of threes”: 3 days for a sharp transection, 3 weeks for an open ragged transection, and 3 months for a closed stretch injury.
Prognosis for recovery depends on the type of injury as well as the treatment. Axonal regeneration occurs at a rate of 1 inch per month, proximally to
distally. Thus, clinical recovery may proceed slowly. Maximal recovery occurs over the course of approximately 1-2 years. Rehabilitation and physical
therapy are important for avoiding the development of muscle contractures that may limit mobility when nerve function has returned. Tendon
transfers may be of assistance if neural function does not completely recover.
PERIPHERAL ENTRAPMENT NEUROPATHIES
Peripheral nerves are subjected to chronic mechanical forces, such as compression, stretching, and friction. These forces may, over time, lead to
peripheral nerve entrapment syndromes, such as carpal tunnel syndrome or ulnar nerve entrapment. Both static and dynamic factors may contribute
to chronic peripheral nerve injury. Static factors include musculotendinous anomalies or inflexible anatomic tunnels that compress peripheral nerves.
Dynamic factors include mobile joints, muscular contraction, or nerve mobility that leads to stretching of a peripheral nerve or increased friction along
its course during movement. Peripheral entrapment neuropathies occur more frequently in upper rather than lower limbs, probably because of the
greater mobility of the arms.
Clinical Findings
Entrapment neuropathies are characterized by weakened muscles, as well as sensory disturbances in the distribution of a single peripheral nerve. In
general, an individual muscle is supplied by one peripheral nerve, and entrapment of that nerve can lead to severe motor findings as well as muscle
atrophy and fasciculations. Marked atrophy is a hallmark of a peripheral nerve lesion. Sensory complaints generally include paresthesias, rather than
pain, in the distribution of the involved nerve. Percussion over the nerve may result in an electric sensation radiating along the nerve and its territory,
known as the Tinel sign. Electrodiagnostic testing is also useful in the diagnosis of peripheral nerve entrapment, and the finding of a nerve conduction
delay at the site of compression is common to all compression neuropathies.
The most common peripheral nerve entrapment syndrome involves the median nerve at the wrist. This condition generally arises from compression of
the median nerve by the transverse carpal ligament and is therefore referred to as carpal tunnel syndrome. Carpal tunnel syndrome occurs with higher
frequency in patients with conditions leading to connective tissue thickening (eg, rheumatoid arthritis, acromegaly, hypothyroidism, pregnancy) and
may be related to repetitive hand or wrist movements. Common presenting symptoms include dysesthetic pain in the hands that is worse at night,
often awaking the patient from sleep. This occurs because many people sleep with flexed wrists, a position that exacerbates median nerve
compression at the wrist. The pain sometimes radiates upward, into the forearm. Patients also complain of numbness on the palmer side of the hand
as well as the first three to three and a half digits. When the ring finger is involved, the sensory disturbance involves only the radial side of the digit.
Patients may complain of decreased grip strength or difficulty grasping small objects as well as atrophy of the abductor pollicis brevis, at the lateral
base of the thumb, due to involvement of the intrinsic hand muscles innervated by the median nerve (first and second lumbricals, opponens pollicis,
abductor pollicis brevis, and flexor pollicis brevis). There is often a positive Tinel sign at the wrist. The Phalen test is a clinical maneuver in which the
patient’s wrists are held in forced flexion for at least 30 seconds and is considered positive if it produces symptoms of median neuropathy in the hand.
Ulnar nerve entrapment is the second most common peripheral nerve entrapment syndrome and most commonly occurs at the elbow. Patients
typically present with upper extremity pain that localizes to the medial aspect of the elbow, as well as paresthesias and numbness of the small finger
and the ulnar half of the ring finger. A Tinel sign is often present at the medial aspect of the elbow: tapping over this area sends electric sensations into
the fourth and fifth digits. The ulnar nerve innervates most of the intrinsic hand muscles, including the adductor pollicis, the first dorsal interosseous,
and the hypothenar muscles, which explains the finding of hand weakness, leading to reduced grip strength and pinch strength. Patients report
dropping things or having trouble opening jars, and on exam, atrophy of the hand intrinsic muscles may be marked, particularly the first dorsal
interosseous and the muscles of the hypothenar eminence. When nerve dysfunction is severe, the hand may take on a “claw hand” appearance.Page 39 / 93
The
patient may also exhibit a Froment sign: when asked to hold a piece of paper between the thumb and index finger, the distal interphalangeal joint will
flex because the patient uses the flexor pollicis longus muscle, innervated by the median nerve, to compensate for lack of abductor pollicis function.
Ulnar nerve entrapment is the second most common peripheral nerve entrapment syndrome and most commonly occurs at the elbow. Patients
typically present with upper extremity pain that localizes to the medial aspect of the elbow, as well as paresthesias and numbness of the small finger
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and the ulnar half of the ring finger. A Tinel sign is often present at the medial aspect of the elbow: tapping over this area sends electric sensations into
the fourth and fifth digits. The ulnar nerve innervates most of the intrinsic hand muscles, including the adductor pollicis, the first dorsal interosseous,
and the hypothenar muscles, which explains the finding of hand weakness, leading to reduced grip strength and pinch strength. Patients report
dropping things or having trouble opening jars, and on exam, atrophy of the hand intrinsic muscles may be marked, particularly the first dorsal
interosseous and the muscles of the hypothenar eminence. When nerve dysfunction is severe, the hand may take on a “claw hand” appearance. The
patient may also exhibit a Froment sign: when asked to hold a piece of paper between the thumb and index finger, the distal interphalangeal joint will
flex because the patient uses the flexor pollicis longus muscle, innervated by the median nerve, to compensate for lack of abductor pollicis function.
The differential diagnosis for peripheral nerve dysfunction includes neuropathies of infectious origin (both bacterial and viral), hereditary conditions
(eg, Charcot-Marie-Tooth disease), neuropathy associated with nutritional deficiency (eg, vitamin B12 deficiency), metabolic or endocrinologic
conditions (eg, diabetes), inflammatory or immune-mediated conditions (eg, polyarteritis nodosa), chronic cervical or lumbar radiculopathy, and toxic
conditions (eg, lead poisoning). It is important to recognize that sensory changes that “split” the ring finger suggest ulnar nerve dysfunction, rather
than C8 radiculopathy. When more than one peripheral nerve is involved, the diagnosis of generalized neuropathy is likely and is typically symmetrical
and bilateral. Decreased amplitude on electrodiagnostic studies (which suggests axonal loss) is characteristic of neuropathy of hereditary or metabolic
origin. Peripheral nerve entrapment, on the other hand, causes damage to the myelin surrounding an axon, thereby slowing conduction velocity but
not affecting amplitude.
Surgical management of peripheral nerve entrapment involves decompressing the involved nerve and should be strongly considered when patients
present with significant motor weakness or muscle atrophy. Additionally, it should be considered when patients fail to improve with medical
treatments. In the case of carpal tunnel syndrome, the transverse carpal ligament is divided, thus relieving compression on the median nerve as it
passes from the wrist into the hand. In the case of ulnar neuropathy, simply freeing the nerve from surrounding scar tissue or hypertrophied
connective tissue (external neurolysis) is usually sufficient. Carpal tunnel release results in excellent relief of symptoms in 80% of patients and partial
relief in another 10%. Similar results are observed for surgical management of ulnar neuropathy.
Nonoperative management strategies include avoidance of repetitive activities that precipitate symptoms or the use of immobilization braces that hold
joints in positions that avoid compression or corticosteroid injections.
PERIPHERAL NERVE TUMORS
Peripheral nerve tumors can be divided into nonneoplastic, benign, and malignant masses. The nonneoplastic masses include traumatic neuromas,
Morton neuromas, and nerve sheath ganglion cysts. Benign masses include neurofibromas, schwannomas (also known as neurilemmomas),
perineuromas, lipofibromatous hamartomas (also known as neural fibrolipomas), nerve sheath myxomas, and granular cell tumors. Malignant masses
include the malignant peripheral nerve sheath tumors.
Neurofibromas can be subdivided into solitary, diffuse, and plexiform varieties. The solitary neurofibroma is the most common benign peripheral
nerve tumor. Axons are incorporated within the neurofibroma along with Schwann cells, collagen matrix, perineurial cells, and fibroblasts. Because
the axons are intermixed within the tumor, the tumor cannot be excised without resecting a portion of the involved nerve. Diffuse neurofibromas and
plexiform neurofibromas are less common than the solitary variety. The diffuse neurofibroma typically involves the skin and subcutaneous tissues.
Plexiform neurofibromas are large, irregular expansions of nerves ranging from small cutaneous nerves to large trunks. Neurofibromas are commonly
found in patients with neurofibromatosis type 1 (von Recklinghausen disease).
Schwannomas are slowly growing lesions made up of Schwann cells in a collagen matrix. Importantly, nerve fascicles run alongside the tumor, rather
than through the tumor as in neurofibromas. Schwannomas are the second most common peripheral nerve tumor. Generally, only mild neurologic
deficits occur, and operative resection can be considered in the setting of pain, paresthesias, or weakness in the distribution of the nerve.
Malignant peripheral nerve sheath tumors are the most common malignant peripheral nerve tumor. Nearly two-thirds arise from neurofibromas in the
setting of neurofibromatosis type 1. The remainder arise either de novo or in patients with a history of prior external-beam radiation.
Clinical Findings
The symptoms of peripheral nerve tumors are generally related to the nerve involved and include weakness, numbness, paresthesias, and pain. MRI
with gadolinium contrast can be useful in imaging the tumors.
than through the tumor as in neurofibromas. Schwannomas are the second most common peripheral nerve tumor. Generally, only mild neurologic
deficits occur, and operative resection can be considered in the setting of pain, paresthesias, or weakness in the distribution of the nerve.
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Malignant peripheral nerve sheath tumors are the most common malignant peripheral nerve tumor. Nearly two-thirds arise from neurofibromas in the
setting of neurofibromatosis type 1. The remainder arise either de novo or in patients with a history of prior external-beam radiation.
Clinical Findings
The symptoms of peripheral nerve tumors are generally related to the nerve involved and include weakness, numbness, paresthesias, and pain. MRI
with gadolinium contrast can be useful in imaging the tumors.
The differential diagnosis of peripheral nerve tumors includes entrapment neuropathies, nonneoplastic masses, radiculopathies, and neuropathies
associated with infection, malnutrition, and metabolic, endocrinologic, inflammatory, immune-mediated, and toxic conditions. Generally, a
symptomatic peripheral nerve tumor will be palpable. Further, the peripheral nerve tumor can occur anywhere along the course of the nerve, whereas
entrapment neuropathies typically occur in defined locations, and the various other neuropathies listed are often diffuse processes. Finally, a
radiculopathy will be symptomatic in the distribution of the nerve root, likely involving several peripheral nerves.
Schwannomas can generally be resected without any neurologic deficit since the nerve fascicles run alongside the tumor and operative resection is
curative. Neurofibromas generally cannot be resected without neurologic deficit since the axons run within the substance of the tumor. Indications for
surgical resection of solitary neurofibromas are large tumor mass, accelerated growth, neurologic deficit, and pain. Plexiform neurofibromas
(excluding superficial ones) can rarely be totally resected and should generally be followed.
Generally, the prognosis for benign peripheral nerve sheath tumors is very good, with improvement in pain, weakness, and paresthesias noted after
resection of solitary lesions. Malignant peripheral nerve sheath tumors require aggressive surgical treatment. The expected 5-year survival is 20%-50%
and neurofibromatosis type 1–associated tumors do not have a worse prognosis than de novo lesions.
James AW, et al: Malignant peripheral nerve sheath tumor. Surg Oncol Clin North Am. 2016;25(4):789–802.
O’Brien M, on behalf of the Guarantors of Brain: Aids to the Examination of the Peripheral Nervous System . 5th ed. Edinburgh, United Kingdom:
Saunders Elsevier; 2010.
Song JW, et al: An outcome study for ulnar neuropathy at the elbow: a multicenter study by the surgery for ulnar nerve (SUN) study group.
Neurosurgery . 2013;72(6):971–981. [PubMed: 23426153]
BRAIN TUMORS
Pablo A. Valdes, MD, PhD, and Timothy R. Smith, MD, PhD
Clinical Presentation
The most common presenting symptoms in patients with intracranial tumors are headaches, seizures, and neurologic deficits. Headaches are the most
common presenting symptom in patients with intracranial tumors (50%). The classical presentation is that of headaches that are worse when waking
up and that are at times severe enough to wake patients from sleep. Headaches in brain tumor patients can result from a variety of factors, including
increased ICP from mass effect in the setting of physiologic elevations in PCO2 during sleep; maneuvers such as straining, coughing, or bending over;
involvement of structures containing pain fibers such as the dura; and visual difficulties through direct or indirect involvement of the optic pathways or
oculomotor and abducens nerves. Headaches can be a helpful initial guide in patients who may also show acute and immediately life-threatening
conditions such as acute hydrocephalus secondary to tumor obstruction of CSF pathways. Acute hydrocephalus is a neurosurgical emergency
requiring prompt intervention (eg, EVD placement). Hemorrhagic tumors with a rapidly expansile mass, patient obtundation, and herniation with
compression of brainstem structures can be a neurosurgical emergency requiring craniotomy for evacuation, resection, and decompression.
Seizures are the most common presenting symptom in patients with low-grade gliomas (> 70%) compared to other intracranial tumors such as high-
grade gliomas (< 20%) and metastatic lesions (< 10%); greater than 25% of patients with intracranial tumors will experience seizures at some point in
their disease course. Seizures can provide useful diagnostic information based on the seizure type and presentation. For example, focal seizures with
a motor component can likely be the result of a mass near or at the contralateral primary motor cortex. Seizures with an epigastric or emotional
component can be likely secondary to a temporal mass or seizures with a clear language disturbance might point to involvement of the language
regions in the left temporoparietal or frontal language regions. Status epilepticus can be a life-threatening condition in the presentation of intracranial
tumors requiring emergent management to achieve immediate medical control in collaboration with the neurologists prior to subsequent surgical
requiring prompt intervention (eg, EVD placement). Hemorrhagic tumors with a rapidly expansile mass, patient obtundation, and herniation with
compression of brainstem structures can be a neurosurgical emergency requiring craniotomy for evacuation, resection, and decompression.
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Seizures are the most common presenting symptom in patients with low-grade gliomas (> 70%) compared to other intracranial tumors such as high-
grade gliomas (< 20%) and metastatic lesions (< 10%); greater than 25% of patients with intracranial tumors will experience seizures at some point in
their disease course. Seizures can provide useful diagnostic information based on the seizure type and presentation. For example, focal seizures with
a motor component can likely be the result of a mass near or at the contralateral primary motor cortex. Seizures with an epigastric or emotional
component can be likely secondary to a temporal mass or seizures with a clear language disturbance might point to involvement of the language
regions in the left temporoparietal or frontal language regions. Status epilepticus can be a life-threatening condition in the presentation of intracranial
tumors requiring emergent management to achieve immediate medical control in collaboration with the neurologists prior to subsequent surgical
intervention.
Neurologic deficit is another common presentation of intracranial tumors that can provide a diagnostic clue as to the localization of the lesion.
Patients presenting with left-sided arm weakness might have a laterally located lesion of the right primary cortex, whereas patients presenting with
left-sided leg weakness might be harboring a right-sided medially located mass affecting the primary motor cortex of the lower extremity homunculus.
Patients presenting with cerebellar signs such as ataxia or dysmetria are likely to have a cerebellar mass. Finally, patients presenting with a rapidly
deteriorating mental status should elicit prompt medical attention and might require emergent intervention because this could point to a rapidly
expansile hemorrhagic mass causing herniation or a metastatic cerebellar hemorrhagic mass causing obstructive hydrocephalus. These are
neurosurgical emergencies requiring prompt neurosurgical attention.
Brain tumor patients can also present with a number of additional symptoms including nausea and vomiting due to elevations in ICP or involvement of
the area postrema in the fourth ventricle; changes in personality in cases affecting the frontal lobe; changes in hearing or balance due to involvement
of the hearing and vestibular systems (cranial nerve VIII); classic bitemporal hemianopsia or homonymous hemianopsia in patients harboring a
pituitary tumor impacting the optic pathways; papilledema due to increased ICP; or endocrinologic disturbances in patients with sellar/suprasellar
lesions. In summary, a careful history with an accurate neurologic exam can provide the clinician with the necessary tools and knowledge to help guide
their subsequent diagnostic workup and urgency of management of the patient with a new intracranial mass.
Diagnostic Workup
A clinical history and exam is the first step to a careful and meticulous workup of the patient with a (tentative) diagnosis of a brain tumor (Table 38–5).
Laboratory studies and radiographic imaging are the next steps in helping further hone the diagnosis of the patient with a history concerning for a new
intracranial mass. Basic laboratory studies including complete blood count, basic metabolic panel, coagulation markers, and, in cases of a highly
suspect pituitary mass, an adequate endocrinologic workup (eg, cortisol, serum sodium, thyroid-stimulating hormone [TSH], luteinizing hormone
[LH], follicle-stimulating hormone [FSH], sodium, prolactin).
Table 38–5.
Brain tumor localizing signs and symptoms.
Location Sign(s)
Frontal lobe1 Impaired intellectual function

Language impairment2, specifically abulia
Impaired gait
Personality changes
Hemiparesis3
Dominant temporal lobe Aphasia

Impaired auditory discrimination
Memory loss
Contralateral superior quadrantanopia
Nondominant temporal lobe Seizures

Visual, auditory, olfactory hallucinations
Uncus CN III palsy

Parietal lobe Impaired sensory perception
Contralateral inferior quadrantanopia
Aphasia
Nondominant temporal lobe Seizures International Medical University
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Visual, auditory, olfactory hallucinations
Uncus CN III palsy
Parietal lobe Impaired sensory perception

Contralateral inferior quadrantanopia
Aphasia
Anosoagnosia4
Occipital lobe Visual deficits
Posterior fossa Posterior headache

Neck stiffness
Opisthotonos
Brainstem Cranial nerve palsies

Long-tract signs
Cerebellopontine angle Unilateral hearing loss

Tinnitus
Vertigo
Facial palsy
Facial anesthesia
Cerebellar signs
Sellar region Endocrime abnormalities

Bitemporal hemianopsia
CN III palsy
Pineal region Parinaud syndrome:

Upgaze palsy
Ptosis
Loss of pupillary light reflex
Retraction-convergence nystagmus
Meningeal infiltration Cranial nerve palsies

Diffuse headache
Meningeal reaction
CN, cranial nerve.
1Usually occurs only if both frontal lobes are involved.
2Occurs only when dominant hemisphere is involved.
3When motor cortex is involved.
4When nondominant temporal lobe is involved.
The next step in the workup of a patient with a newly suspected intracranial process is the use of noncontrast CT given its speed, availability, and
affordability to help provide an initial screening test. An initial CT can note any acute process (hemorrhage, hydrocephalus) and also provide
information regarding the presence of an intracranial mass that would substantiate further imaging (ie, MRI). Noncontrast CT of the brain provides
excellent imaging of hemorrhagic masses, bony structures, and bony infiltration, but poor description regarding soft tissue lesions (eg, gliomas,
meningiomas, metastases). If CT imaging notes a likely mass lesion, the next step is MRI imaging of the brain with and without gadolinium contrast (to
Chapter 38: Neurosurgery, Timothy R. Smith Page 43
help highlight tumors that have significant uptake of this contrast agent such as high-grade gliomas, meningiomas, and metastases). The typical / 93
MRI
sequences provide high-resolution images for the visualization of soft tissue structures and composition of the brain. In brain tumor patients, MRI can
provide exquisite details regarding tumor composition including location, size, cellularity, cystic components, hemorrhage, surrounding edema,
vascularity, necrosis, and association with surrounding soft tissue structures. MRI sequences, in addition to the standard structural sequences (ie, T1-
When nondominant temporal lobe is involved.
The next step in the workup of a patient with a newly suspected intracranial process is the use of noncontrast CT given its speed, availability, and
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affordability to help provide an initial screening test. An initial CT can note any acute process (hemorrhage, hydrocephalus) and also provide
information regarding the presence of an intracranial mass that would substantiate further imaging (ie, MRI). Noncontrast CT of the brain provides
excellent imaging of hemorrhagic masses, bony structures, and bony infiltration, but poor description regarding soft tissue lesions (eg, gliomas,
meningiomas, metastases). If CT imaging notes a likely mass lesion, the next step is MRI imaging of the brain with and without gadolinium contrast (to
help highlight tumors that have significant uptake of this contrast agent such as high-grade gliomas, meningiomas, and metastases). The typical MRI
sequences provide high-resolution images for the visualization of soft tissue structures and composition of the brain. In brain tumor patients, MRI can
provide exquisite details regarding tumor composition including location, size, cellularity, cystic components, hemorrhage, surrounding edema,
vascularity, necrosis, and association with surrounding soft tissue structures. MRI sequences, in addition to the standard structural sequences (ie, T1-
weighted and T2-weighted), can provide further information to help guide diagnosis and urgency of treatment. Diffusion images can provide clues
regarding cellularity and density of tumor or help distinguish between a tumor and abscess, where the latter would require urgent surgical
intervention. Perfusion imaging can be used to assess vascularity of tumors as well as help assess tumor recurrence versus treatment-induced changes
in the posttreatment period. Spectroscopy can further provide information regarding more subtle molecular changes (eg, lactate contents) to further
hone the preoperative presumed diagnosis and assessment of tumor recurrence. All these sequences can help further guide the preoperative
diagnosis when distinguishing between a likely brain tumor and an abscess (eg, significant diffusion restriction), in which the latter is a neurosurgical
emergency. MRI can provide a good estimate of the preoperative diagnosis (eg, high-grade glioma, meningioma, vestibular schwannoma) of an
intracranial lesion, but histology remains the gold standard.
In some special cases, the use of cerebral angiography is warranted for both diagnostic and, more frequently, treatment reasons. In cases of a
hemorrhagic mass in which MRI can be equivocal, angiography can be used to help identify a possible vascular nidus to identify a hemorrhagic lesion
as an arteriovenous malformation rather than a tumor, which requires a different surgical approach and expertise. In cases of highly vascular tumors
such as hemangiopericytomas, some meningiomas, or hemangioblastomas, preoperative angiography can aid in identification of feeding vessels
and/or embolization to diminish operative risks and difficulty.
The differential of possible intracranial lesions is extensive and can include both intra-axial (ie, within the brain parenchyma) and extra-axial (ie,
external to the brain parenchyma) lesions. The most common intra-axial tumors are primary brain tumors (eg, high- and low-grade gliomas) and
metastases. The most common extra-axial tumors are pituitary tumors, meningiomas, and schwannomas. The differential diagnosis of new
intracranial masses is extensive and should be narrowed to ascertain those lesions requiring acute, immediate management (eg, ruptured intracranial
aneurysm, hemorrhagic cerebellar tumors with brainstem compression or acute hydrocephalus, brain abscess) and those requiring elective
management and workup. The differential diagnosis can be narrowed based on history (patient demographics, chronology of symptoms, past medical
history, and specific neurologic deficits), physical exam, and imaging characteristics, including location, size, number, and sequence-specific
characteristics. The list is extensive, including neoplastic processes with primary intracranial lesions such as gliomas, meningiomas, schwannomas,
pituitary tumors, and lymphoma; metastatic lesions; infectious lesions such as abscesses; vascular masses such as aneurysms, arteriovenous
malformations, and cavernous malformations; nonneoplastic processes such as multiple sclerosis, AIDS, dermoid cysts, arachnoid cysts, epidermoid
cysts; and hemorrhagic or nonhemorrhagic infarct.
The frequency, types, and presentations of intracranial tumors differ greatly between the adult and pediatric populations. In the adult population, the
most common intracranial tumors are metastases, supratentorial gliomas, pituitary tumors, and meningiomas. In the pediatric population, metastases
to the brain are rare, and the most common primary brain tumors are infratentorial medulloblastomas, juvenile pilocytic astrocytomas, and
ependymomas; more commonly seen in the pediatric population are entities such as embryonal tumors (atypical teratoid/rhabdoid tumor) and pineal
tumors (germ cell tumors, such as germinomas, and nongerminomatous germ cell tumors, such as choriocarcinomas, teratomas, and yolk sac
tumors). Here, we will focus our discussion on the most common adult pathologies; pituitary tumors are discussed in a separate chapter.
The clinician will a carefully gathered history, physical exam, and imaging workup to help identify the most likely diagnosis(es) to guide management.
As such, the use of neurologic expertise working in conjunction with both the neurologist and neurosurgeon can help the general practitioner
maximize resource utilization and optimize treatment for these patients.
Tumor Types
A. Intra-axial Tumors
The 2016 World Health Organization (WHO) Classification of Tumors of the CNS provided the community with a conceptual and practical advance in
classifying CNS tumors using both novel molecular parameters and traditional histology to define tumor entities. In this new classification, genotype
trumps histologic phenotype when providing a diagnosis for tumor entities. Here, will use the newer classification but intermittently refer to the older,
more familiar system to help guide the reader.
1. Diffuse gliomas and glioblastoma
Tumor Types
A. Intra-axial Tumors
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The 2016 World Health Organization (WHO) Classification of Tumors of the CNS provided the community with a conceptual and practical advance in
classifying CNS tumors using both novel molecular parameters and traditional histology to define tumor entities. In this new classification, genotype
trumps histologic phenotype when providing a diagnosis for tumor entities. Here, will use the newer classification but intermittently refer to the older,
more familiar system to help guide the reader.
1. Diffuse gliomas and glioblastoma
Diffuse gliomas encompass the more commonly known WHO grades II to III low- and high-grade gliomas of astrocytic and/or oligodendrocytic lineage.
Gliomas account for > 70% of all primary brain tumors (glioblastoma > 60% and diffuse gliomas > 10%). Gliomas are now divided into IDH mutant, IDH
wild-type, and not otherwise specified (NOS; which is usually reserved for cases in which tissue diagnosis is limited due to factors such as limitations in
tissue quantity or access to molecular studies). Diffuse WHO grade II and III gliomas are mostly IDH mutant. IDH mutant diffuse gliomas are further
separated into IDH mutant with a 1p/19q codeletion, which encompass the majority of the traditional oligodendrogliomas, and IDH mutant (without a
1p19q codeletion) with an ATRX loss/TP53 mutation, which are mostly diffuse astrocytomas. Patients harboring IDH mutations have a better prognosis
(> 5 years) compared to those with an IDH wild-type glioma (generally 1-2 years).
Diffuse gliomas constitute the more commonly known low-grade gliomas and are slow-growing lesions within the white matter with modest to low
cellularity and no significant endothelial proliferation or necrosis (unlike glioblastoma). WHO grade II IDH mutant astrocytomas are usually non–
contrast enhancing on T1-weighted imaging but hyperintense on T2-weighted and FLAIR imaging. WHO grade II IDH mutant diffuse gliomas with 1p19q
codeletion encompass the more commonly known oligodendrogliomas with the classic perinuclear halos or “fried egg” appearance on histologic
evaluation. On MRI, these lesions are usually non–contrast enhancing on T1-weighted imaging but hyperintense on T2-weighted and FLAIR imaging.
They may demonstrate hypointense regions on MRI that may correspond to hyperdense areas on CT denoting intratumoral calcifications. IDH mutant
lesions can demonstrate contrast enhancement on T1-weighted imaging usually upon malignant transformation to WHO grade III or IV lesions (eg,
secondary glioblastoma). Current treatment varies among institutions but generally includes a combination of watchful waiting, surgery, and/or
chemotherapy with procarbazine/lomustine/vincristine or temozolomide with survival rates of approximately 5-20 years. Patients with diffuse low-
grade lesions usually do not undergo radiation.
Glioblastomas are generally IDH wild-type (90%; de novo or primary glioblastoma) tumors seen in older patients (> 55 years of age), whereas IDH
mutant glioblastomas are much less common (10%; secondary glioblastoma arising from previously low-grade gliomas) and are seen in younger
patients (< 30 years of age). Despite current maximal therapy including surgery, chemotherapy, radiation, and tumor-treating fields, patients have an
overall survival of < 2 years. These lesions are highly invasive along white matter tracts and vasculature and demonstrate high cellularity, endothelial
proliferation, nuclear atypia, mitotic figures, and necrosis on histologic evaluation. MRI demonstrates a pattern of ring enhancement on T1-weighted
imaging with a central area of T1-weighted hypointensity that corresponds to the necrotic core of the tumor with surrounding T2-weighted and FLAIR
hyperintensity spanning from the tumor core corresponding to tumor edema and an infiltrative component (Figure 38–13). The mainstay of treatment
for glioblastoma involves maximal surgical resection followed by radiation, temozolomide, and more recently, the use of tumor-treating fields, with
overall median survival of 21 months.
Figure 38–13.
Magnetic resonance imaging of a deep right temporal mass proven to be a high-grade glioma. A : Fluid-attenuated inversion recovery imaging
demonstrating a mass with significant surrounding white matter edema. B : T1-weighted contrast-enhanced image demonstrating smaller enhancing
portion of the mass.
Figure 38–13.
Magnetic resonance imaging of a deep right temporal mass proven to be a high-grade glioma. A : Fluid-attenuated inversion recovery imaging
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demonstrating a mass with significant surrounding white matter edema. B : T1-weighted contrast-enhanced image demonstrating smaller enhancing
portion of the mass.
B. Extra-axial Tumors
1. Meningiomas
Meningiomas are the most common primary intracranial tumors, accounting for one-third of all primary CNS tumors with a pathologically confirmed
prevalence of 97.5 in 100,000 in the United States and a peak incidence at 45 years of age. Nevertheless, this is a likely underestimate of the actual
prevalence because imaging of asymptomatic meningiomas is 0.9% in adults, and autopsy studies suggest an even higher prevalence of up to 3% in
persons over 60 years of age. Meningiomas are generally benign, extra-axial, slow-growing lesions arising from the arachnoid caps cells of the
meninges, with lower grade meningiomas more common in women and higher grade more common in men. Ionizing radiation is a known risk factor in
patients undergoing prior cranial irradiation (eg, patients with history of irradiated childhood brain tumors). Certain genetic associations have been
noted as well. Specifically, neurofibromatosis type 2 greatly increases the risk of meningiomas and schwannomas; other syndromes also increase risk,
including neurofibromatosis type 1, Gorlin syndrome, Li-Fraumeni syndrome, von Hippel-Lindau syndrome, and Cowden disease.
Meningiomas are categorized as WHO grade I (benign, 70%), II (atypical, 30%), or III (anaplastic, ∼1%) and include multiple histologic subtypes (eg,
meningothelial, fibrous, psammomatous, angiomatous). They are characterized at the histologic level by densely packed sheets of cells (similar to
arachnoid cells), psammoma bodies (whorls of calcium and collagen), intranuclear cytoplasmic pseudo-inclusions, and Orphan Annie nuclei (nuclei
with central clearing from peripheral migration of chromatin).
Meningiomas can appear slightly hyperdense to normal on brain CT (∼60%), contain intratumoral calcification (> 20%), and demonstrate hyperostosis
of the skull (5%). MRI is the modality of choice, with meningiomas appearing as extra-axial masses with a dural base or “tail” (secondary to dural
thickening rather than invasion of tumor) (Figure 38–14). A vascular cleft sign suggests the extra-axial nature of these lesions. They are typically
homogeneous and well circumscribed and have isointense signal on T1-weighted imaging, intense and homogeneous contrast enhancement on T1-
weighted postcontrast imaging, and iso- to hyperintense signal on T2-weighted imaging at the tumor core, and in more invasive areas of tumor
surrounding brain parenchyma, T2-weighted hyperintense signal suggests edema and possible tumor invasion (60%). Nevertheless, despite all these
characteristics, it is virtually impossible to distinguish between meningioma grades on preoperative imaging.
Figure 38–14.
Sagittal magnetic resonance imaging demonstrating a contrast-enhancing dural-based lesion (arrow) proven to be a meningioma at time of resection.
characteristics, it is virtually impossible to distinguish between meningioma grades on preoperative imaging.
Figure 38–14.
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Sagittal magnetic resonance imaging demonstrating a contrast-enhancing dural-based lesion (arrow) proven to be a meningioma at time of resection.
Most meningiomas are asymptomatic, but symptomatic ones can present with seizures, headaches, or focal neurologic deficits (eg, contralateral arms
weakness) either directly as space-occupying lesions or indirectly through peritumoral vasogenic edema. As such, treatment with antiepileptics and
steroids should be reserved for patients with seizures or symptomatic patients with imaging evidence of peritumoral edema, respectively. Surgery is
the mainstay of treatment for meningiomas, with the goal of surgery entailing complete removal of the lesion, surrounding dura, and any involved
bone. The Simpson grading scale provides a clinical risk assessment of recurrence by relating the degree of resection with the risk of recurrence,
subsequently helping guide the need for adjuvant treatment. Radiation can serve many roles; for example, it can be used in patients after initial surgery
with high risk of recurrence, as primary therapy in patients ineligible for surgery, or for treatment of recurrent disease without a one-size-fits-all
approach. At this time, there are no chemotherapy regimens with demonstrated efficacy against meningiomas.
C. Brain Metastases
Brain metastases (BMs) are the most common intracranial tumors, with a yearly incidence of approximately 200,000 cases per year in the United States,
affecting up to one-third of adults with cancer and occurring more frequently in adults in their fifth to seventh decades of life. Historically, BM had a
dismal prognosis, with median survival of < 6 months, but recent advances with earlier diagnosis, newer treatments, and better understanding of
molecular subtypes has led to significant improvements in overall survival. Specifically, in an era of improving systemic therapies, molecular
understanding, and personalized medicine, the diagnosis-specific graded prognostic assessment has been used as a means for more accurate patient
stratification. This specific stratification reveals the marked heterogeneity in outcomes and important difference in prognostication for the different
BM subgroups.
The five most common primary cancers that metastasize to the CNS in adults are lung (20%), melanoma (7%), renal (7%), breast (5%), and colorectal
(2%). In the era of immunologic therapies, the natural disease history can be altered, and the risk for BM differs for particular cancers (eg, human
epidermal growth factor receptor 2 [HER2]–positive breast cancer, melanoma, and ALK-rearranged non–small-cell lung cancer), with an incidence of
BM of up to 40% in HER2-positive breast cancer and overall improved survival. Treatment of melanoma with immune checkpoint inhibitors and
targeted treatments can provide survival advantage for systemic disease and CNS control.
Patients will typically present with any combination of symptoms including but not limited to headaches, seizures, changes in mental status, focal
neurologic deficits, nausea, and vomiting. Nevertheless, up to 60% of patient are asymptomatic at the time of imaging confirmed BM. Of note,
histologic characteristics are specific to the primary cancer.
CT is the first-line imaging modality to visualize a mass-occupying lesion with varying iso-, hypo-, or hyperdense features and variable associated
vasogenic edema. Variable contrast enhancement is noted on CT, including solid, ring, punctuate, or diffuse enhancement. MRI is more sensitive than
CT at identifying BM with typical iso- to hypointense T1-weighted signal; uniform, solid, ring, punctuate, and variable patterns of contrast enhancement
are noted, with contrast-enhanced sequences used as standard for detection of smaller metastases. Typically, BM demonstrate T2-weighted and FLAIR
hyperintense signal with variable degree of peritumoral edema. These lesions are classically found at the gray-white matter interface but can be found
Chapter 38: Neurosurgery, Timothy R. Smith Page 47and
anywhere in the brain (Figure 38–15). Specific primary BMs demonstrate a propensity for hemorrhage including melanoma, renal cell carcinoma, / 93
choriocarcinoma. In cases of intratumoral hemorrhage, tumors will show associated CT hyperdense regions and intrinsic hyperintense signal on T1-
weighted precontrast imaging.
CT is the first-line imaging modality to visualize a mass-occupying lesion with varying iso-, hypo-, or hyperdense featuresInternational Medical University
and variable associated
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vasogenic edema. Variable contrast enhancement is noted on CT, including solid, ring, punctuate, or diffuse enhancement. MRI is more sensitive than
CT at identifying BM with typical iso- to hypointense T1-weighted signal; uniform, solid, ring, punctuate, and variable patterns of contrast enhancement
are noted, with contrast-enhanced sequences used as standard for detection of smaller metastases. Typically, BM demonstrate T2-weighted and FLAIR
hyperintense signal with variable degree of peritumoral edema. These lesions are classically found at the gray-white matter interface but can be found
anywhere in the brain (Figure 38–15). Specific primary BMs demonstrate a propensity for hemorrhage including melanoma, renal cell carcinoma, and
choriocarcinoma. In cases of intratumoral hemorrhage, tumors will show associated CT hyperdense regions and intrinsic hyperintense signal on T1-
weighted precontrast imaging.
Figure 38–15.
Sagittal magnetic resonance image demonstrating multiple contrast-enhancing lesions (arrows) in the infra- and supratentorial spaces. Biopsy
demonstrated metastatic adenocarcinoma.
Treatment of BMs involves a multidisciplinary approach that includes a combination of surgery, stereotactic radiation, whole-brain radiation therapy
(WBRT), and/or chemotherapy. Surgery has been typically reserved for patients with a single BM in a favorable location that is large in size (generally >
3 cm) with considerable mass effect and/or edema; patients with a good performance status and limited or absent systemic disease; radioresistant
primary cancer (eg, renal cell, melanoma); tissue diagnosis to distinguish necrosis from true recurrence; and cerebellar mass effect causing
compression and possible hydrocephalus. More recent data with advances in surgical techniques have shown efficacy in surgical resection of multiple
BMs, including resection of a dominant lesion among two to three lesions followed by radiation. As such, data for resection of more than one lesion
seem to suggest an advantage in carefully selected patients. Patients can undergo a combination of WBRT and or stereotactic radiation depending on
location, number of lesions, and size (generally < 3 cm). With the advent of molecular classification and new targeted therapies, patients with BM
undergo specific chemotherapy regimens based on their primary cancer with varied response and highly promising recent results (eg, control of
melanoma).
General Surgical Considerations
Every tumor requires an individualized, tailored approach and treatment, but several general principles can help guide and inform management.
Surgeons need to decide for each case whether the goal of surgery is biopsy, subtotal resection, or gross total resection, with gross total resection
being the gold standard in cases where it is deemed safe, providing the best survival benefit in patients. Tumor resection requires careful
consideration of a number of key factors including: (1) tumor size; (2) location; (3) gross, radiographic, and pathologic characteristics; (4) sensitivity to
radiation; and importantly, (5) medical and neurologic status of the patient.
Timing is important in preoperative planning, such as in patients with rapid deterioration (eg, elevated ICP may require prompt intervention either by
an EVD in cases of acute hydrocephalus or decompression and resection in cases of a rapidly expansile hemorrhagic tumor). Generally, tumor surgery
can usually be done on an elective to semi-elective basis depending on neurologic presentation. Some tumors can even be followed long term without
any surgical or treatment intervention (eg, small, stable meningiomas).
Modern approaches for tumor resection use a number of intraoperative tools, including head immobilization using a Mayfield head holder;
intraoperative imaging including ultrasound, CT, and MRI; intraoperative navigation or frameless stereotaxy, which matches preoperativePage
Chapter 38: Neurosurgery, Timothy R. Smith imaging
48 /with
93
the patient’s intraoperative location; intraoperative electrophysiologic monitoring to help identify functioning eloquent versus noneloquent brain,
which can aid in delineating functional and safe borders during resection; and more recently, the use of intraoperative fluorescent dyes, which help
visualize tumors. The approaches have resulted in significant improvement in gross total resection rates. The main goal of brain tumor surgery is
Timing is important in preoperative planning, such as in patients with rapid deterioration (eg, elevated ICP may require prompt intervention either by
an EVD in cases of acute hydrocephalus or decompression and resection in cases of a rapidly expansile hemorrhagic tumor). Generally, tumor surgery
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can usually be done on an elective to semi-elective basis depending on neurologic presentation. Some tumors can even be followed long term without
any surgical or treatment intervention (eg, small, stable meningiomas).
Modern approaches for tumor resection use a number of intraoperative tools, including head immobilization using a Mayfield head holder;
intraoperative imaging including ultrasound, CT, and MRI; intraoperative navigation or frameless stereotaxy, which matches preoperative imaging with
the patient’s intraoperative location; intraoperative electrophysiologic monitoring to help identify functioning eloquent versus noneloquent brain,
which can aid in delineating functional and safe borders during resection; and more recently, the use of intraoperative fluorescent dyes, which help
visualize tumors. The approaches have resulted in significant improvement in gross total resection rates. The main goal of brain tumor surgery is
maximizing the extent of tumor resection while minimizing damage to surrounding normal brain and vascular structures, and the specific surgical
approaches vary based on tumor type (eg, transsphenoidal for a functioning pituitary microadenoma vs retrosigmoid for a cerebellopontine angle
meningioma). Generally, except in rare instances, maximizing the extent of tumor resection provides patients with the greatest survival advantage
across tumor pathologies.
Postoperatively patients are observed in an ICU or step-down unit usually overnight with close neurologic monitoring. Steroids are used in some cases
depending on associated edema and surgeon preference. Anticonvulsants are used in cases where brain parenchyma has been dissected/contacted
and are continued for an extended period only in patients with a history of seizures. Postoperative MRI is done routinely within 48 hours to provide a
baseline to evaluate for residual tumor and future evaluation of tumor recurrence.
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Buckner J, Giannini C, Eckel-Passow J, et al: Management of diffuse low-grade gliomas in adults: use of molecular diagnostics. Nat Rev Neurol .
2017;13(6):340–351. [PubMed: 28497806]
Garcia CR, Slone SA, Pittman T, et al: Comprehensive evaluation of treatment and outcomes of low-grade diffuse gliomas. PloS One .
2018;13(9):e0203639. [PubMed: 30235224]
Hervey-Jumper SL, Berger MS: Maximizing safe resection of low- and high-grade glioma. J Neuro-Oncol . 2016;130(2):269–282.
Louis DN, Perry A, Reifenberger G, et al: (2016). The 2016 World Health Organization classification of tumors of the central nervous system: a
summary. Acta Neuropathol . 2016;131(6):803–820. [PubMed: 27157931]
Rogers L, Barani I, Chamberlain M, et al: Meningiomas: knowledge base, treatment outcomes, and uncertainties. A RANO review. J Neurosurg .
2016;122(1):4–23.
Stupp R, Mason WP, van den Bent MJ, et al: Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med .
2005;352(10):987–996. [PubMed: 15758009]
Wen PY, Chang SM, Van den Bent MJ, et al: Response assessment in neuro-oncology clinical trials. J Clin Oncol. 2017;35(21):2439–2449. [PubMed:
28640707]
Wiemels J, Wrensch M, Claus EB: Epidemiology and etiology of meningioma. J Neuro-Oncol . 2010;99(3):307–314.
TUMORS OF THE SPINE AND SPINAL CORD

Introduction
Tumors of the spine and spinal cord are uncommon in the general population but are an important consideration in the evaluation of patients
presenting with neck and/or back pain, with or without associated radicular symptoms, sensorimotor deficits, and bowel or bladder dysfunction. An
estimated 15% of primary CNS tumors are intraspinal, and most of these are benign. Surgery, radiotherapy, and chemotherapy, alone or in
conjunction, remain the best current management of spinal tumors.
In addition to gender and age of presentation, localization of the lesion to the cervical, thoracic, lumbar, or sacrococcygeal spine aids in refining the
differential diagnosis because certain tumors demonstrate a predilection for particular regions of the spinal column. Spinal tumors are differentiated
based on their location relative to three anatomic compartments: extradural, intradural-extramedullary, and intradural-intramedullary. Extradural
tumors are located outside of the thecal sac and arise either from the osseous spine or epidural space. Intradural-extramedullary tumors occur within
the thecal sac but are outside of the neural tissues of the spinal cord. These tumors most commonly develop from the leptomeninges or nerve roots.
Tumors of the spine and spinal cord are uncommon in the general population but are an important consideration in the evaluation of patients
presenting with neck and/or back pain, with or without associated radicular symptoms, sensorimotor deficits, and bowel or bladder dysfunction. An
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estimated 15% of primary CNS tumors are intraspinal, and most of these are benign. Surgery, radiotherapy, and chemotherapy, alone or in
conjunction, remain the best current management of spinal tumors.
In addition to gender and age of presentation, localization of the lesion to the cervical, thoracic, lumbar, or sacrococcygeal spine aids in refining the
differential diagnosis because certain tumors demonstrate a predilection for particular regions of the spinal column. Spinal tumors are differentiated
based on their location relative to three anatomic compartments: extradural, intradural-extramedullary, and intradural-intramedullary. Extradural
tumors are located outside of the thecal sac and arise either from the osseous spine or epidural space. Intradural-extramedullary tumors occur within
the thecal sac but are outside of the neural tissues of the spinal cord. These tumors most commonly develop from the leptomeninges or nerve roots.
Intramedullary tumors are found within the spinal cord and originate from either the spinal cord parenchyma or pia mater.
Symptoms are more commonly produced by compression than direct invasion of the spinal cord or nerve roots. Classically, the pain associated with
neoplasms is unremitting, worse in the supine position, and more noticeable at rest or in bed. Hence, the patient may wake up at night due to pain.
Although the progression of symptoms can be insidious, radicular pain, motor weakness, paresthesias, or anesthesia can frequently occur as a result
of nerve compression. Long tract myelopathic findings such as ataxia, hyperreflexia, spasticity, fasciculations, sensorimotor loss, or sphincter
dysfunction can be caused by spinal cord compression. Recognized unusual findings include muscle wasting or hyporeflexia, referred pain, autonomic
changes such as in Horner syndrome, and Brown-Séquard hemi-cord syndrome. Fracture and deformity causing axial pain often occur as well and may
be the presenting complaint.
Extradural tumors frequently involve the osseous spine, and so most typically present with axial pain, often increased by motion or Valsalva maneuver.
Signs and symptoms of neural compression occur secondarily. Intradural-extramedullary tumors present most commonly with motor deficits and
other long tract disturbances. Intramedullary tumors can demonstrate an insidious succession of symptoms including neuralgic pain that can progress
to myelopathy.
Diagnosis
A. Radiographic Evaluation
MRI, specifically contrast-enhanced T1-weighted sequences, is the standard imaging modality to assess spinal tumors (Figure 38–16). Nearly all
intramedullary lesions demonstrate contrast uptake, and the resolution that MRI provides is typically sufficient for determination of margins and
infiltration. The addition of angiography is indicated for the suspicion of a vascular pathology.
Figure 38–16.
Sagittal T1-weighted image with contrast of the upper thoracic spinal cord demonstrating an intradural, intramedullary lesion found on biopsy to be a
high-grade glioma.
Figure 38–16.
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Sagittal T1-weighted image with contrast of the upper thoracic spinal cord demonstrating an intradural, intramedullary lesion found on biopsy to be a
high-grade glioma.
If an MRI is not obtainable, then myelography can still provide excellent structural detail. Fusiform cord widening, a dumbbell-shaped deformity, and
complete blockage are classic findings suggesting the presence of a mass lesion. Plain, contrast-enhanced, and postmyelography CT imaging can aid in
the assessment of bony spine anatomy. Nuclear scintigraphy is used primarily in the identification of skeletal metastases. Catheter angiography can be
employed in the evaluation of suspected vascular lesions. Plain x-rays of the spine have limited utility in the initial assessment of spinal tumors.
Findings such as enlarged intervertebral foramina and interpedicular spaces or bone erosion with scalloped edges suggest the presence of an
enlarging mass.
B. Laboratory Analysis
Lumbar puncture can provide additional clues to the presence of a spinal cord tumor. Elevated CSF protein is present in approximately 95% of cases,
although CSF glucose is normal. Xanthochromia and the presence of fibrinogen causing clotting can also occur. Specific CSF and serum
immunocytochemical tests can aid the diagnosis of specific neoplasms.
C. Differential Diagnosis
Extradural lesions compose an estimated 55%-60% of all spinal tumors, and a vast majority of these are metastatic lesions via hematogenous
dissemination along Batson venous plexus. The most common primary sources of vertebral metastases are lung, breast, and prostate.
Benign tumors of the osseous spine are very rare and may be incidental findings or may cause pain, radiculopathy, myelopathy, spinal instability, or
deformity. Biopsy is used at times, and appropriate management may be observation, as is common in the case of eosinophilic granuloma; ablative, as
can be performed for osteoid osteoma; or surgical. Primary benign spine tumors can be classified using the Enneking system.
Approximately 5% of bone malignancies involve the spine, the four most common being osteosarcoma, chondrosarcoma, Ewing sarcoma, and
chordoma. Survival varies significantly by pathology. The high rates of recurrence, limited survival duration, and functional morbidity associated with
these tumors support the aggressive multimodal strategies typically used in the treatment of these tumors. In some cases, complete resection with
negative margins, or en bloc resection, is possible. In other cases, surgical resection may serve to decrease tumor burden, thereby improving the
efficacy of adjuvant treatments such as chemotherapy and radiotherapy. At times, surgical intervention is necessary for functional indications, either
for neural decompression or spinal stabilization.
Only 0.5% of tumors involving the spinal column are primary neoplasms. Of the intradural tumors, an estimated 70% are extramedullary and
Chapter 38: Neurosurgery, Timothy R. Smith consist
Page 51 / 93
mainly of schwannoma, neurofibroma, and meningioma. The nerve sheath tumors, schwannoma and neurofibroma, are usually benign and can
demonstrate a typical dumbbell-shaped appearance, caused by compression from the neuroforamina through which they sometimes pass. They are
an important consideration when deviation of the pleural reflection on x-ray suggests a posterior mediastinal mass. Schwannomas are well-
Approximately 5% of bone malignancies involve the spine, the four most common being osteosarcoma, chondrosarcoma, Ewing sarcoma, and
chordoma. Survival varies significantly by pathology. The high rates of recurrence, limited survival duration, and functional morbidity associated with
these tumors support the aggressive multimodal strategies typically used in the treatment of these tumors. In some cases, complete resection with
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negative margins, or en bloc resection, is possible. In other cases, surgical resection may serve to decrease tumor burden, thereby improving the
efficacy of adjuvant treatments such as chemotherapy and radiotherapy. At times, surgical intervention is necessary for functional indications, either
for neural decompression or spinal stabilization.
Only 0.5% of tumors involving the spinal column are primary neoplasms. Of the intradural tumors, an estimated 70% are extramedullary and consist
mainly of schwannoma, neurofibroma, and meningioma. The nerve sheath tumors, schwannoma and neurofibroma, are usually benign and can
demonstrate a typical dumbbell-shaped appearance, caused by compression from the neuroforamina through which they sometimes pass. They are
an important consideration when deviation of the pleural reflection on x-ray suggests a posterior mediastinal mass. Schwannomas are well-
differentiated and typically grossly resectable; however, neurofibromas cannot be resected completely from their parent nerve. In the setting of type 1
neurofibromatosis, suspicion of multiple neurofibromas, meningioma, and ependymoma should be heightened. Schwannoma, neurofibroma, and
meningioma multiplicity is associated with type 2 neurofibromatosis. Meningiomas are seen most often in the thoracic spines of middle-aged women
and arise from persistent arachnoid cap cells.
Intramedullary tumors compose only approximately 10% of all spinal tumors and arise most commonly in the cervical segment of the spinal cord. Glial
tumors are the most common of these, with ependymomas occurring twice as frequently as astrocytomas in adults. In children, the relationship is
reversed, with astrocytomas being twice as common as ependymomas. Myxopapillary ependymomas form at the conus medullaris and filum terminale
and are a very common tumor to be found at this location.
Treatment
Generally, primary tumors of the spinal column are best treated by complete resection, if possible. In the vertebral column, en bloc resection is
frequently the goal of surgery, whereas with any involvement of the spinal cord, gross total resection is the desired treatment. Symptomatic deformity
or spinal instability warrants consideration for surgical fixation, as functional deterioration profoundly affects survival.
The role of surgery in epidural disease can include decompressing the spinal cord, obtaining tissue for histologic diagnosis, mechanical stabilization,
and decreasing tumor burden to increase the effectiveness of radiotherapy. The role of surgery and its interplay with chemoradiation depends on
many factors, including the type of lesion, its location, and the patient’s prognosis, and the decision regarding surgery is generally made on an
individual basis by a multidisciplinary treatment team.
In 2016, the WHO updated a comprehensive classification of neoplasms affecting the CNS based on molecular parameters and histology that guides
therapy and prognosis in most intramedullary tumors. Overall, functional outcome depends heavily on the severity and duration of neurologic
symptoms at the time of presentation, whereas survival outcome depends largely on the pathology in question and, less so, on the ability to gain
adequate surgical resection.
Intradural-extramedullary tumors are generally best treated by surgical resection; outcomes appear to depend heavily on extent of resection, although
preservation of preexisting neurologic function remains the primary objective in surgery. Radiation and chemotherapy are increasingly important in
the management of intradural tumors, especially with recurrence or multiloculated lesions. Determination of a dissection plane between tumor and
spinal cord is the initial aim of any resection procedure. Overall, prognosis is excellent in nearly all cases of spinal intradural-extramedullary
neoplasms, unless paraplegia is the presenting condition. In contrast, recurrence of infiltrative neoplasms is very common, and progression to
paralysis is common. Progressive neurologic deterioration demands particular focus on facilitating appropriate surgical therapy.
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29455369]
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Ozawa H, et al: Spinal dumbbell tumors: an analysis of a series of 118 cases. J Neurosurg Spine . 2007;7(6):587–593. [PubMed: 18074682]
Thakur NA, et al: Benign tumors of the spine. J Am Acad Orthop Surg . 2012;20(11):715–724. [PubMed: 23118137]
Zadnik PL, et al: Spinal cord tumours: advances in genetics and their implications for treatment. Nat Rev Neurol . 2013;9(5):257–266. [PubMed:
23528542]
PITUITARY TUMORS
The pituitary gland is involved in hormonal regulation of the body and is composed of the anterior pituitary (adenohypophysis), posterior pituitary
(neurohypophysis), and functionally the pars intermedia separating the two lobes. The anterior pituitary secretes prolactin, adrenocorticotropin
hormone (ACTH), TSH, LH, FSH, and growth hormone (GH); meanwhile, the posterior pituitary is a repository of the hypothalamic hormones oxytocin
and antidiuretic hormone (ADH; vasopressin).
Pituitary tumors account for 10%-20% of all primary intracranial tumors, making them the third most common after gliomas and meningiomas. The
most common are pituitary adenomas. They have a peak incidence at 40-50 years of age, with functioning (ie, secreting) tumors more frequent in
young adults and nonfunctioning (ie, nonsecreting) tumors more common in older adults, with a slight female preponderance. They are almost always
benign tumors developing from the anterior pituitary (adenohypophysis) and rarely from the posterior pituitary (neurohypophysis).
Pituitary adenomas are classified based on histology, endocrine function, and size. Secreting tumors release supraphysiologic levels of hormones that
result in distinct clinical syndromes and account for almost two-thirds of cases (meanwhile, nonsecretory tumors account for the remaining third). The
most common adenomas are prolactin secreting (32%-66%), GH secreting (8%-16%), and adrenocorticotropin secreting (2%-6%).
Pituitary tumors can be classified based on size, with approximately 50% of tumors < 10 mm on MRI (known as microadenomas) and approximately
50% of tumors > 10 mm (known as macroadenomas). The new WHO 2017 classification of pituitary adenomas focuses on an adenohypophyseal cell
lineage as well as clinical and pathologic prognostic parameters for predicting high risk and aggressive adenomas. The three main adenohypophyseal
cell differentiation pathways are corticotrophs, somatotrophs/lactotrophs/mammosomatotrophs/thyrotrophs, and gonadotrophs.
Pituitary tumors can present with a variety of symptoms depending on size, location, and hormonal status (ie, secreting vs nonsecreting). Compression
and/or mass effect of an adenoma can lead to a variety of symptoms including headaches, visual field deficits, ophthalmoplegia, and facial pain. The
optic chiasm is located superior to the pituitary gland in the majority of cases (80%) such that larger adenomas can grow superiorly, leading to contact,
elevation, compression, and/or displacement of the optic chiasm, ultimately leading to the classical bitemporal hemianopsia in patients from
compression of the decussating nasal fibers. Visual compromise secondary to optic nerve/chiasm compression is usually an indication for treatment
because these can progress to eventual loss of acuity and blindness. Pituitary adenomas also commonly invade the cavernous sinus laterally up to
complete 360-degree encasement of the surrounding cavernous internal carotid, which can lead to oculomotor (cranial nerve [CN] III) and abducens
(CN VI) palsies, with patients presenting with complaints of diplopia and ophthalmoplegia on neurologic examination. Significant mass effect and
encasement of the cavernous sinus can also lead to facial pain due to V1-V2 compression or even proptosis/chemosis due to occlusion of the
cavernous sinus. Invasion of the cavernous sinus can be graded and help guide surgical treatment and planning.
Secretory tumors can present with endocrinologic symptoms related to hypersecretion of a specific hormone. Hypersecretion of prolactin may include
diminished libido, infertility, and an amenorrhea-galactorrhea syndrome in women and impotence in men. Levels greater than 300 ng/mL are usually
associated with a prolactinoma, whereas modestly high levels of 150 ng/mL or less are typically more likely in cases of pituitary stalk interruption (ie,
“stalk effect” due to release of tonic inhibition of hypothalamic dopamine on prolactin-producing cells). In cases of extremely elevated prolactin, one
should ensure serial dilutions to avoid a false negative by the so-called “hook effect,” which results from excess prolactin saturating the laboratory
assay causing a false-negative read.
Hypersecretion of GH causes acromegaly in adults and gigantism in children with classical physical changes such as skeletal and soft tissue
overgrowth, macroglossia, nerve entrapment syndromes, and associated increased morbidity and mortality due to cardiomyopathy, sleep apnea, and
glucose intolerance. Patients can notice changes in shoe sizes as adult or inability to wear a wedding ring, with an overall insidious onset of the
disease.
Hypersecretion of ACTH by adenomas accounts for 65% of cases of hypercortisolism, leading to Cushing disease. The signs and symptoms of
hypercortisolism are known as Cushing syndrome, with characteristic findings such as moon-shaped face, excess hair growth, easy bruising,
menstrual irregularity, hypertension, buffalo hump, obesity, abdominal striae, glucose intolerance, osteoporosis, poor wound healing, amenorrhea,
should ensure serial dilutions to avoid a false negative by the so-called “hook effect,” which results from excess prolactin saturating the laboratory
assay causing a false-negative read.
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Hypersecretion of GH causes acromegaly in adults and gigantism in children with classical physical changes such as skeletal and soft tissue
overgrowth, macroglossia, nerve entrapment syndromes, and associated increased morbidity and mortality due to cardiomyopathy, sleep apnea, and
glucose intolerance. Patients can notice changes in shoe sizes as adult or inability to wear a wedding ring, with an overall insidious onset of the
disease.
Hypersecretion of ACTH by adenomas accounts for 65% of cases of hypercortisolism, leading to Cushing disease. The signs and symptoms of
hypercortisolism are known as Cushing syndrome, with characteristic findings such as moon-shaped face, excess hair growth, easy bruising,
menstrual irregularity, hypertension, buffalo hump, obesity, abdominal striae, glucose intolerance, osteoporosis, poor wound healing, amenorrhea,
impotence, and hyperpigmentation. Cushing disease patients have up to a two- to fivefold increased morbidity and mortality if left untreated.
Hypersecretion of TSH is rare (< 1%), with classic findings of hyperthyroidism including tachycardia, weight loss, heat intolerance, anxiety, and tremor.
Rarely, pituitary adenomas can also hypersecrete gonadotropins (LH and FSH) with a usually silent presentation except in women, in which they can
present with amenorrhea and infertility.
Patients can present with excessive thirst and urination as a result of stalk compression and loss of ADH. This is known as diabetes insipidus, in which
patients have an inability to concentrate their urine, resulting in dehydration and hypernatremia.
Apoplexy, or acute pituitary failure, is considered a neurosurgical emergency and usually results from hemorrhage and/or necrosis in preexisting
adenomas, leading to an acute presentation with severe headache, visual changes, and ophthalmoplegia; changes in mental status; and Addisonian
crises. These patients require acute medical management of their endocrinologic and electrolyte status (eg, steroid administration, diabetes insipidus)
to avoid cardiovascular collapse. Moreover, surgical decompression is often indicated to prevent blindness from compression of the optic apparatus.
The differential diagnosis of sellar and suprasellar lesions is broad. It is fundamental to distinguish among the various possibilities by means of a
detailed history, physical, MRI, and endocrinologic testing. The differential includes, but is not limited to, pituitary adenoma, craniopharyngioma,
Rathke cleft cyst, meningioma, optic pathway glioma, dermoid, epidermoid, lymphoma, carcinoma, pituicytoma, abscess, aneurysm, neurosarcoidosis,
Langerhans cell histiocytosis, hypophysitis, and metastases.
Diagnostic Workup
Initial evaluation should include careful note of symptoms related to hyper- or hyposecretion of hormones or compression of surrounding structures.
Ideally, a visual field examination with formal visual field testing should be performed. Imaging should be done to help ascertain size, location, and
likely preoperative diagnosis. Pituitary tumors are sellar and/or suprasellar masses (specifically with adenomas) that vary in their CT features
depending on presence of hemorrhage and cystic and necrotic components. Solid adenomas without cysts or hemorrhage are typically isodense to
brain and rarely harbor calcifications (the latter are a more common feature of craniopharyngiomas). MRI should be obtained with and without
contrast with thin cuts through the sella in the coronal and sagittal planes to better identify the size, configuration, and extent of invasion of tumor and
location of normal gland (Figure 38–17). MRI shows a typically isointense lesion but can vary with a more heterogeneous pattern with hypointense
regions (cyst) or hyperintense area (hemorrhage) on T1-weighted imaging; solid components demonstrate contrast enhancement. T2-weighted
imaging demonstrates isointense signal but can vary if a more heterogeneous lesion with hyperintense (cyst) or hypointense (hemorrhage) regions is
present. Gradient echo/SWI images can provide a more sensitive means of assessing hemorrhagic components.
Figure 38–17.
Magnetic resonance imaging in (A) sagittal plain and (B) coronal plain of a pituitary macroadenoma.
present. Gradient echo/SWI images can provide a more sensitive means of assessing hemorrhagic components.
Figure 38–17.
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Magnetic resonance imaging in (A) sagittal plain and (B) coronal plain of a pituitary macroadenoma.
Laboratory testing should include direct or indirect evaluation of anterior pituitary hormones including prolactin, fasting morning cortisol, free T4,
TSH, insulin-like growth factor-1 (IGF-1), GH, LH, FSH, and testosterone. Studies should also include serum sodium and osmolality as well as urine
osmolality or specific gravity to confirm diagnosis. In cases with concern for hypercortisolism, additional studies can be performed including 24-hour
urinary free cortisol, low and high dexamethasone suppression tests, and ACTH levels to help confirm location of pathology (eg, pituitary adenoma,
adrenal adenoma or carcinoma, or ACTH-producing pulmonary malignancy). IGF-1 is the gold standard for diagnosis of GH-secreting tumors since
levels are relatively stable and is a better confirmatory test than the more volatile levels of GH.
Treatment
The mainstay of treatment involves medical and/or surgical approaches that depend on endocrinologic status, location, size, and the patient’s health
status. Nonsecreting adenomas without compressive symptoms can be managed conservatively and followed with serial imaging. Nonsecreting
adenomas causing compressive symptoms and secreting adenomas causing compressive and/or purely endocrinologic disturbance are usually
treated surgically. The most common approach is a microscopic or endoscopic transsphenoidal approach (ie, into the nasal cavity, through the
sphenoid sinus, and to the sella). This approach provides a more minimally invasive approach to treat these lesions. In some cases with large tumors
where a transsphenoidal approach does not provide optimal resection (eg, extensive suprasellar or lateral extension), a craniotomy might be
indicated. Prolactinomas are the exception because they can usually respond well to medical treatment alone.
Treatment of hypersecreting adenomas depends on the hormone secreted. Prolactinomas can usually first be treated medically with dopamine
agonists such as bromocriptine or cabergoline to decrease size and even result in cure. Most prolactinomas respond well, but 10%-20% of cases do
not, in which circumstances surgical treatment is indicated. Other medical options include somatostatin analogs to reduce GH levels and associated
symptoms or also control production of TSH. In cases were medical management alone is not indicated or sufficient, patients will undergo surgical
treatment. Finally, radiation can be an option, usually in recurrent or aggressive tumors.
Postoperatively patients might require hormonal supplementation, including but not limited to steroids (eg, hydrocortisone), desmopressin (DDAVP),
and levothyroxine. Diabetes insipidus following pituitary surgery can occur in up to 30% of cases in the immediate postoperative setting, although a
majority will eventually recover function. Some patients maybe undergo a triple-phase response, comprising an early transient diabetes insipidus,
followed by antidiuresis and hyponatremia, and finally resolving into a permanent diabetes insipidus.
Inoshita N, Nishioka H. The 2017 WHO classification of pituitary adenoma: overview and comments. Brain Tumor Pathol . 2018;35(2):51–56. [PubMed:
29687298]
Kopczak A, Renner U, Stalla KG: Advances in understanding pituitary tumors. F1000Prime Rep . 2014;6:5. [PubMed: 24592317]
Melmed S: Pathogenesis of pituitary tumors. Nat Rev Endocrinol . 2011;7(5):257–266. [PubMed: 21423242]
Molitch ME: Diagnosis and treatment of pituitary adenomas: a review. JAMA . 2017;317(5):516–524. [PubMed: 28170483]
PEDIATRIC NEUROSURGERY
Kevin T. Huang, MD, and Timothy R. Smith, MD, PhD

Melmed S: Pathogenesis of pituitary tumors. Nat Rev Endocrinol . 2011;7(5):257–266. [PubMed: 21423242]
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Molitch ME: Diagnosis and treatment of pituitary adenomas: a review. JAMA . 2017;317(5):516–524. [PubMed: 28170483]
PEDIATRIC NEUROSURGERY
CONGENITAL MALFORMATIONS
Craniospinal Dysraphisms
Craniospinal dysraphisms encompass a group of disorders that arise from improper neural tube closure during embryonic development. These may
be classified based on whether they are open or closed (with closed defects covered with skin), the location of the lesion, or the embryologic basis for
the malformation.
A. Myelomeningocele
Myelomeningocele, also known as spina bifida, occurs in approximately 1 in every 1200-1400 live births. Its incidence has sharply declined in the
developed world with prenatal folate supplementation, but cases persist because causative factors include both genetic and environmental factors.
The condition arises during primary neurulation from failure of neural tube closure, which occurs between postfertilization days 21 and 28 in humans.
The result is a child born with a cutaneous defect in the lumbosacral region, with an exposed sheet of neural tissue known as a neural placode. This is
accompanied by several neural dysfunctions and malformations, including congenital dysfunction of the lumbosacral nerves (and associated lower
limb paralysis and lack of bowel/bladder function), hydrocephalus, and descended cerebellar tonsils (Chiari II malformation, see below).
The diagnosis of a neural tube defect can be suspected prenatally with an elevated maternal serum alpha-fetoprotein and confirmed by in utero
imaging such as a maternal-fetal MRI or ultrasound. Pregnant mothers who have inadequate folate intake or who have other children with neural tube
defects are at increased risk for giving birth to a child with a myelomeningocele. Immediately following birth, care of the defect involves placing the
baby prone (avoiding pressure on the placode) and keeping the defect moist, followed by prompt closure of the defect. Other considerations include
urologic attention for bladder dysfunction and monitoring for any developing hydrocephalus.
B. Closed Neural Tube Defects and Tethered Cord Syndrome
Closed neural tube defects include a wide spectrum of disorders and arise from problems with disjunction, secondary neurulation, or postneurulation
events. Types of closed neural tube defects include dermal sinus tracts, spinal lipomas, neurenteric cysts, sacral dysgenesis, and diastematomyelia.
They can be suspected in children with certain superficial markers of the lower back, such as a distinct tuft of hair, sacral dimple, visible sinus tract,
palpable mass, a prominent port-wine stain, hemangioma, hyper- or hypopigmented macule, or a significantly deviated gluteal cleft. Presence of
abnormal cutaneous stigmata should warrant further screening with imaging.
Many of these abnormalities, with some as subtle as a fatty or thickened filum terminale, can tether the spinal cord in an abnormally low position,
leading to tethered cord syndrome. It should be noted that the normal spinal cord conus terminates at approximately the level of the L1-2 interspace.
Children may initially be asymptomatic, but as the child grows, the tethering can cause traction of the spinal cord. Neuronal dysfunction subsequently
ensues with symptoms such as back or leg pain, worsening lower extremity motor and sensory function, decline in bladder and bowel function,
worsening lower extremity orthopedic deformities, and progressive scoliosis. Surgical repair is often recommended early in life in order to prevent the
onset or halt the progression of neurologic symptoms.
Other specific malformations may require surgical intervention for other reasons as well. Dermal sinus tracts, which can directly communicate the CNS
to the outside world, require closure to prevent infection. These can also occur in the cranial region and similarly require repair. Neurenteric cysts,
remnants of involuted endodermal material often located intradurally in the ventral thoracic spinal canal, may require resection to prevent
enlargement and spinal cord impingement. Lipomyelomeningoceles, involving mesenchymal growth developing into a lipoma involved with the spinal
cord itself, may require more complex debulking and detethering procedures. Diastematomyelia, or split-cord malformation, may involve resection of
any bony spurs separating the two hemicords and possibly joining of the two thecal sacs.
C. Cranial Dysraphisms
Many cranial dysraphisms are incompatible with life, but when CNS contents are present at birth herniating through a congenital skull defect, they are
known as an encephalocele. These herniated tissues include a mixture of neural tissue, meninges, and CSF. Prognosis for the children is dependent on
the amount of tissue contained within the encephalocele, and encephaloceles are frequently associated with other congenital anomalies. Surgical
repair involves removal of the sac, perseveration of any underlying functional tissue, and closure to prevent egress of CSF.
enlargement and spinal cord impingement. Lipomyelomeningoceles, involving mesenchymal growth developing into a lipoma involved with the spinal
cord itself, may require more complex debulking and detethering procedures. Diastematomyelia, or split-cord malformation, may involve resection of
any bony spurs separating the two hemicords and possibly joining of the two thecal sacs. Access Provided by:
C. Cranial Dysraphisms
Many cranial dysraphisms are incompatible with life, but when CNS contents are present at birth herniating through a congenital skull defect, they are
known as an encephalocele. These herniated tissues include a mixture of neural tissue, meninges, and CSF. Prognosis for the children is dependent on
the amount of tissue contained within the encephalocele, and encephaloceles are frequently associated with other congenital anomalies. Surgical
repair involves removal of the sac, perseveration of any underlying functional tissue, and closure to prevent egress of CSF.
Arachnoid Cysts
Arachnoid cysts are developmental anomalies that form intradurally, are lined with arachnoid matter, and contain CSF. These cysts most commonly
occur in the middle cranial fossa and retrocerebellar region. The brain may be shifted by the arachnoid cyst, but the overall brain volume is usually
close to normal. Arachnoid cysts are often asymptomatic, incidental findings that do not require further intervention, and they typically do not expand
over time. Occasionally, however, they can present with either symptoms of local compression nearby neural tissue and/or gradual enlargement over
time. When clearly symptomatic, they can be surgically treated with cyst fenestration, with cystoperitoneal shunting available for refractory cases.
Chiari Malformations
Chiari malformations encompass a group of congenital malformations of the hindbrain and posterior fossa. Although four different types have been
reported, only two are commonly encountered.
Chiari I malformations are most common and are defined as when the cerebellar tonsils are herniated below the level of the foramen magnum (Figure
38–18). It is important to note that many of these are asymptomatic and do not require treatment. Moreover, presenting complains are often
nonspecific, such as headaches, which can be difficult to attribute specifically to the Chiari malformation. Symptoms are thought to arise from
crowding of the foramen magnum. Symptoms thought to be more specific to a Chiari malformation include occipital headaches that worsen with
increased ICP (eg, when coughing or laughing), evidence of syrinx formation in the spinal cord (eg, weakness, numbness, motor regression, or
progressive scoliosis), and any evidence of brainstem dysfunction (eg, central sleep apnea). The workup should include imaging of the spine in
addition to the brain to assess for the presence of a syrinx. Surgical decision making can be challenging because symptoms can be nonspecific, but
patients with symptoms clearly referable to the lesion should be treated. Surgical correction consists of bony decompression of the back of the
foramen magnum (suboccipital craniectomy), with the possible addition of a C1 laminectomy and expansile duraplasty for additional decompression.
Figure 38–18.
Sagittal T2-weighted magnetic resonance imaging scan of a Chiari I malformation showing typical peg-like appearance of cerebellar tonsils and
associated syringomyelia.
Chiari II malformations are similarly identified as low-lying cerebellar tonsils herniating through the foramen magnum but arise from the abnormal
CNS development secondary to a myelomeningocele. In contrast to Chiari I patients, these patients are more prone to having symptomatic
hydrocephalus and are seen as part of a constellation of other CNS developmental abnormalities. Although symptoms can be more complex, surgical
Figure 38–18.
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Sagittal T2-weighted magnetic resonance imaging scan of a Chiari I malformation showing typical peg-like appearance of cerebellar tonsils and
associated syringomyelia.
Chiari II malformations are similarly identified as low-lying cerebellar tonsils herniating through the foramen magnum but arise from the abnormal
CNS development secondary to a myelomeningocele. In contrast to Chiari I patients, these patients are more prone to having symptomatic
hydrocephalus and are seen as part of a constellation of other CNS developmental abnormalities. Although symptoms can be more complex, surgical
treatment similarly involves posterior fossa decompression. Chiari III and IV malformations are very rare. Chiari III malformations include features of
Chiari II malformation as well as an occipital encephalocele. Chiari IV malformations are characterized by severe cerebellar hypoplasia without an
encephalocele.
Craniosynostosis
Craniosynostosis refers to the premature fusion of one or more cranial sutures. When this occurs, bone growth is restricted in a direction
perpendicular to the fused suture, and there is compensatory growth at other sites. The result is a misshapen head, which may worsen as the child
grows and possibly restrict brain growth. This can typically be detected on physical examination, where a distinct ridge can be palpated along the
abnormally fused suture, one of the classic abnormal head shapes can be observed when viewing the child’s head, and the head circumference may be
reduced. On careful observation, these findings can often be detected immediately upon birth.
Certain sutures are encountered in synostosis more commonly than others, with sagittal and unilateral coronal synostosis occurring much more
commonly than the exceedingly rare lambdoid synostosis. The head shapes they produce are named in Greek for what they resemble, ranging from
the elongated head of sagittal synostosis (scaphocephaly or dolichocephaly), to the skewed head shape of unilateral coronal synostosis
(plagiocephaly), to the triangle shape of metopic synostosis (trigonocephaly). Notably, craniosynostosis should not be confused with the more
common, nonsynostotic condition, positional plagiocephaly, which arises from an infant lying preferentially on one side and will typically resolve with
conservative management.
Although most cases of craniosynostosis are sporadic and involve a single suture, multiple-suture craniosynostosis occurs in certain genetic
syndromes, such as Crouzon or Apert syndrome. In such cases, treatment is indicated not only for cosmetic reasons, but also because the multiple-
suture synostosis is restricting brain growth and causing elevated ICPs.
Operative repair of craniosynostosis is dependent on the age of the child. When performed early enough, endoscopic removal of the fused suture can
allow a return to a more normal shape, but cases that are either more severe or present later may require more extensive cranial vault reconstruction.
HYDROCEPHALUS
Disturbances in CSF circulation or absorption result in hydrocephalus. At a basic level, hydrocephalus can be classified into two types: communicating
or noncommunicating (sometimes referred to as “obstructive”). In noncommunicating hydrocephalus, there is an identifiable blockage ofPage
Chapter 38: Neurosurgery, Timothy R. Smith CSF flow
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within the ventricular system (eg, by a tumor). In communicating hydrocephalus, no such barrier
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presumed to be blocked at the level of the arachnoid granulations. It is rare for hydrocephalus to be caused by overproduction of CSF (eg, by a choroid
plexus tumor). Hydrocephalus can be found in any stage of life but is a particularly salient issue in children because neonatal hemorrhage or
Operative repair of craniosynostosis is dependent on the age of the child. When performed early enough, endoscopic removal of the fused suture can
allow a return to a more normal shape, but cases that are either more severe or present later may require more extensive cranial vault reconstruction.
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HYDROCEPHALUS
Disturbances in CSF circulation or absorption result in hydrocephalus. At a basic level, hydrocephalus can be classified into two types: communicating
or noncommunicating (sometimes referred to as “obstructive”). In noncommunicating hydrocephalus, there is an identifiable blockage of CSF flow
within the ventricular system (eg, by a tumor). In communicating hydrocephalus, no such barrier is clearly identifiable, and CSF absorption is
presumed to be blocked at the level of the arachnoid granulations. It is rare for hydrocephalus to be caused by overproduction of CSF (eg, by a choroid
plexus tumor). Hydrocephalus can be found in any stage of life but is a particularly salient issue in children because neonatal hemorrhage or
meningitis/encephalitis are common causes.
Hydrocephalus can cause elevations in ICP that may manifest in different ways depending on the age of the child. In neonates and infants whose
anterior fontanelle is still open, untreated hydrocephalus will present with a tense or bulging fontanelle, apneic and bradycardic episodes,
engorgement of the scalp veins, forced downgaze (ie, upward gaze palsy), splayed cranial sutures, rapid increases in head circumference, irritability,
poor head control, and poor oral intake. In children with a closed cranial vault whose fontanelle has closed, untreated hydrocephalus will present with
symptoms of intracranial hypertension including lethargy or excessive sleepiness, papilledema, headache, nausea, vomiting, gait disturbance,
increased fussiness, or upward gaze palsy.
Several surgical options can be considered in the treatment of hydrocephalus. The most common CSF diversionary procedure remains
ventriculoperitoneal shunting, which installs tubing that allows the cerebral ventricles to drain into the peritoneal cavity by way of a control valve.
Other types of shunts may drain into other locations including the right atrium (ventriculoatrial shunt) or pleural cavity (ventriculopleural shunt).
Ventriculosubgaleal shunting may be performed as a temporary measure in infants. Recently, there has also been increased interest in the use of
endoscopic third ventriculostomy with choroid plexus cauterization, which involves creating an alternative pathway for CSF flow through fenestration
of the floor of the third ventricle. Although traditionally this was applied mostly to cases of noncommunicating hydrocephalus, there is increased
interest in its application to cases of communicating hydrocephalus as well.
Shunt failure or infection may manifest with signs and symptoms of acute intracranial hypertension. Importantly, ventricular enlargement may or may
not be present in shunt failure. Prompt treatment of acute hydrocephalus and/or shunt failure is indicated to prevent irreversible neurologic injury
including herniation, blindness, or death.
PEDIATRIC CENTRAL NERVOUS SYSTEM TUMORS
Brain tumors are the most common solid tumors of childhood. The locations and types of tumors in the pediatric population differ from those in
adults, and most tumors in children under the age of 12 occur in the infratentorial space. In neonates and infants, brain tumors may manifest with
nonspecific findings, and mass effect from a tumor may not be clinically evident initially due to a compliant skull and an open fontanelle. In young
children, a primary brain tumor may present with symptoms related to intracranial hypertension such as headache, nausea, and vomiting. Papilledema
may be evident on funduscopic examination. Older children more often present with focal neurologic signs and symptoms. When a posterior fossa
brain tumor is diagnosed, preoperative imaging of the brain and spine should be performed whenever possible to evaluate for drop metastases in the
spinal canal.
Medulloblastoma
Medulloblastomas compose approximately 20% of all pediatric brain tumors and 30% of all posterior fossa tumors in children. They are thought to
arise from residual embryonic tissue and appear as hyperdense lesions in the region of the fourth ventricle that enhance following contrast
administration (Figure 38–19). Although traditionally they have been classified histologically, increasingly it is being viewed as useful to classify them
by molecular expression, with consensus of at least four distinct subgroups: WNT, SHH, group 3, and group 4.
Figure 38–19.
Head computed tomography demonstrating large mass (medulloblastoma) within the fourth ventricle causing ventricular dilation.
by molecular expression, with consensus of at least four distinct subgroups: WNT, SHH, group 3, and group 4.
Figure 38–19.
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Head computed tomography demonstrating large mass (medulloblastoma) within the fourth ventricle causing ventricular dilation.
When encountered, treatment involves complete or near-complete surgical resection with postoperative craniospinal radiation in children old enough
to tolerate it (generally those age 3 years or older). Patients who are classified as high risk are typically treated with chemotherapy as well. Because of
the increased morbidity of radiation in children less than 3 years of age, chemotherapy can be used to delay the radiation dose in such young patients.
The 5-year overall survival is reported at between 65% and 75% but is lower in patients with diffuse disease, with postsurgical residuals, or presenting
at younger ages.
Cerebellar Astrocytoma/Pilocytic Astrocytomas
Cerebellar astrocytomas (> 75% of which are pilocytic astrocytomas histologically) account for approximately 20% of all pediatric brain tumors. The
peak age of presentation is 10 years. The characteristic appearance on imaging studies is an enhancing mural nodule with a surrounding cyst. The goal
of treatment is complete surgical resection, as patients with a gross total resection have a 90% long-term survival rate without any need for additional
adjuvant therapies.
Ependymoma
Ependymomas arise from the ependymal cells lining the ventricular system and can arise anywhere along the neuraxis with an ependymal surface. In
the pediatric population, 90% of ependymomas are intracranial, and of these, two-thirds are located in the posterior fossa. Ependymoma cells are
known to migrate through the CSF and can often be found in distal sites within the CNS, underscoring the importance of full neuraxis imaging to
properly stage the disease. Treatment usually consists of tumor resection and postoperative focal radiation.
Brainstem Glioma
Brainstem gliomas are a heterogeneous group of tumors of varying histology, biologic behavior, and prognosis. Diffuse brainstem gliomas represent
up to 80% of all brainstem gliomas and carry the worst prognosis. Affected children typically present with a relatively short clinical history of unilateral
or bilateral cranial neuropathies, progressive ataxia, gait abnormality, and long tract signs. On MRI studies, diffuse brainstem gliomas appear as
nonenhancing, T1-hypointense, T2-hyperintense masses that expand the brainstem (typically the pons) (Figure 38–20). Diagnosis of a diffuse
brainstem tumor can be made on the basis of imaging alone, and biopsy is usually not recommended. Radiation therapy and steroids may improve
symptoms but have not been shown to prolong survival, as these tumors are universally fatal, with a median survival of 8-10 months.
Figure 38–20.
Magnetic resonance imaging appearance of a diffuse pontine glioma.

brainstem tumor can be made on the basis of imaging alone, and biopsy is usually not recommended. Radiation therapy and steroids may improve
symptoms but have not been shown to prolong survival, as these tumors are universally fatal, with a median survival of 8-10 months.
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Figure 38–20.
Magnetic resonance imaging appearance of a diffuse pontine glioma.
Other types of brainstem gliomas are associated with a better prognosis. Dorsally exophytic brainstem tumors are characterized by slow growth with
gradual onset of symptoms and can sometimes be excised with limited morbidity. Moreover, nondiffuse gliomas such as focal intrinsic and dorsally
exophytic brainstem gliomas are usually histologically lower grade (WHO grade I or II) lesions.
Tumors of Infancy
Brain tumors that affect infants include medulloblastoma, central neuroblastoma, supratentorial primitive neuroectodermal tumor (PNET),
pineoblastoma, and atypical teratoid/rhabdoid tumor. These are all high-grade tumors with a propensity to spread throughout the CSF.
Medulloblastoma, central neuroblastoma, supratentorial PNET, and pineoblastomas are all considered PNETs with similar histologies. Atypical
teratoid/rhabdoid tumors are usually found in the posterior fossa and are associated with a poor prognosis, as most children die within 1 year of
diagnosis.
Pineal Region Tumors
Tumors arising in the region of the pineal gland compose 3%-8% of pediatric brain tumors. Histologically, pineal region tumors are most often germ
cell tumors such as germinomas, teratomas (mature and immature), embryonal cell carcinomas, choriocarcinomas, and endodermal sinus tumors.
Germinomas are the most common pineal region tumors and demonstrate a gender predilection for males. Pineal parenchymal tumors such as
pineocytomas or pineoblastomas occur less frequently. Pineal tumors can compress the cerebral aqueduct and cause hydrocephalus. Patients may
present with Parinaud syndrome consisting of impaired upgaze, convergence-retraction nystagmus, lid retraction, convergence paralysis, pupillary
dilatation, and light-near dissociation. Complete neuraxis imaging should be performed because drop metastases can occur via CSF pathways. Serum
and CSF should be tested for tumor markers that may be secreted by germ cell tumors including placental alkaline phosphatase, alpha-fetoprotein,
and beta-human chorionic gonadotropin. Radiation therapy with or without chemotherapy is the mainstay of treatment for germ cells tumors.
Dysembryoplastic Neuroepithelial Tumors
Dysembryoplastic neuroepithelial tumor is a low-grade cortical based tumor with a median age of presentation of 7 years. They appear as superficial
cystic tumors in the temporal or frontal lobes. On MRI, they are hypointense on T1-weighted sequences, hyperintense on T2-weighted sequences, do
not enhance, and are often described as having a “bubbly” appearance. Patients with these tumors typically present with a long history of intractable
complex partial seizures and have a normal neurologic exam. Gross total resection of the tumor is curative and usually eliminates seizures.
Sellar Tumors
Tumors that arise in the region of the sella turcica and pituitary gland in children include pituitary adenomas and craniopharyngiomas. Pituitary
adenomas are relatively rare among children. Craniopharyngiomas, however, represent 6%-9% of all pediatric brain tumors and are the most common
Dysembryoplastic neuroepithelial tumor is a low-grade cortical based tumor with a median age of presentation of 7 years. They appear as superficial
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cystic tumors in the temporal or frontal lobes. On MRI, they are hypointense on T1-weighted sequences, hyperintense on T2-weighted sequences, do
not enhance, and are often described as having a “bubbly” appearance. Patients with these tumors typically present with a long history of intractable
complex partial seizures and have a normal neurologic exam. Gross total resection of the tumor is curative and usually eliminates seizures.
Sellar Tumors
Tumors that arise in the region of the sella turcica and pituitary gland in children include pituitary adenomas and craniopharyngiomas. Pituitary
adenomas are relatively rare among children. Craniopharyngiomas, however, represent 6%-9% of all pediatric brain tumors and are the most common
nonglial intracranial masses in children. On imaging, craniopharyngiomas appear as cystic tumors that originate from a suprasellar location.
Calcification is commonly visible on CT scan and can be helpful in differentiating craniopharyngiomas from other types of tumors. They typically
present either with signs of local mass effect (eg, headaches, visual disturbances, and/or noncommunicating hydrocephalus) or symptoms of
endocrine dysfunction such as growth failure, diabetes insipidus, hypothyroidism, or menstrual dysfunction. A thorough endocrine workup is
warranted in patients with suspected craniopharyngioma. Surgical resection is the mainstay of treatment, but extent of resection is often limited by the
tumor’s location, and thus radiotherapy is often employed as either primary or adjuvant therapy. Postoperatively, patients should be watched for signs
of diabetes insipidus or hypothalamic insufficiency.
Choroid Plexus Tumors
Choroid plexus tumors include choroid plexus papillomas and choroid plexus carcinomas. They represent 2%-4% of all pediatric brain tumors and
present as an intraventricular mass that avidly enhances with contrast. For choroid plexus papillomas, prognosis is generally favorable and can
typically be treated with gross total resection alone. Choroid plexus carcinomas are malignant tumors for which the average age of diagnosis is 2 years.
They are commonly already disseminated at the time of diagnosis, and prognosis is generally poor. Treatment consists of surgical resection, radiation
therapy, and possibly chemotherapy.
CEREBROVASCULAR DISEASE IN CHILDREN
Aneurysms and Vascular Malformations
Children may present with a variety of intracranial vascular malformations such as arteriovenous malformations (AVMs), venous angiomas, capillary
telangiectasias, and cavernous malformations. AVMs are congenital malformations that often go undetected until they present with an
intraparenchymal hemorrhage, which can range in severity from severe headaches to life-threatening increased ICP. AVMs are usually treated with
surgery in the pediatric age group but may occasionally be treated with stereotactic radiation or embolization techniques. Cavernous malformations
can also present with intracerebral hemorrhage but are more likely to primarily present with seizures. Surgical resection is indicated in symptomatic
patients. Although relatively common in the adult population, cerebral aneurysms are rare in the pediatric population. Depending on the size and
configuration of the aneurysm, these lesions may be treated by surgical clipping or endovascular coiling or managed conservatively.
Vein of Galen Malformations
Vein of Galen malformations are congenital vascular malformations characterized by extensive arterial feeders draining into an enlarged vein of Galen.
Although these malformations are also known as vein of Galen aneurysms, they represent arteriovenous fistulae. Newborns can present with high-
output cardiac failure due to the arteriovenous shunting. The extensive arteriovenous shunting can also produce a steal effect and result in cerebral
ischemia and infarction. Moreover, hydrocephalus is a common finding due to compression of the cerebral aqueduct by the malformation. The
prognosis may be poor for patients diagnosed in early infancy with heart failure but is better for those diagnosed later in life. Treatment usually
involves endovascular embolization of feeding arteries.
Moyamoya Disease
Moyamoya disease is an idiopathic vasculopathy that leads to progressive occlusion of one or both internal carotid arteries. Secondary to this
phenomenon, there is angiogenesis about the stenotic point, leading to the formation of a capillary network that looks like a “puff of smoke”
(translated to “moyamoya” in Japanese) on cerebral angiogram. The disease can also involve the proximal middle and anterior cerebral arteries.
Children with moyamoya disease present with ischemic events that may be provoked by straining or hyperventilation. Refractory headaches, seizures,
and alternating hemiplegia are also associated with moyamoya disease. Moyamoya is treated with surgical revascularization to improve blood flow via
direct or indirect bypass procedures.
SPASTICITY
Chapter 38: Neurosurgery, Timothy R. Smith Pagewith
Spasticity in children is most often due to cerebral palsy, and several surgical interventions exist for treatment. In determining whether a child 62 / 93
hypertonia will benefit from surgical intervention, it is important to assess whether dystonia is present, as well the ambulatory potential of the child,
and to what extent the underlying spasticity is useful for the child in terms of providing strength and postural support. In addition to medication,
orthopedic procedures, and periodic injections that are used to treat spasticity, the neurosurgical treatments for spasticity include placement of an
(translated to “moyamoya” in Japanese) on cerebral angiogram. The disease can also involve the proximal middle and anterior cerebral arteries.
Children with moyamoya disease present with ischemic events that may be provoked by straining or hyperventilation. Refractory headaches, seizures,
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and alternating hemiplegia are also associated with moyamoya disease. Moyamoya is treated with surgical revascularization to improve blood flow via
direct or indirect bypass procedures.
SPASTICITY
Spasticity in children is most often due to cerebral palsy, and several surgical interventions exist for treatment. In determining whether a child with
hypertonia will benefit from surgical intervention, it is important to assess whether dystonia is present, as well the ambulatory potential of the child,
and to what extent the underlying spasticity is useful for the child in terms of providing strength and postural support. In addition to medication,
orthopedic procedures, and periodic injections that are used to treat spasticity, the neurosurgical treatments for spasticity include placement of an
intrathecal baclofen pump and selective dorsal rhizotomy (SDR).
An intrathecal baclofen pump entails inserting a catheter into the intrathecal space and connecting it to a programmable subcutaneous pump.
Depending on at what spinal level the catheter tip is placed, upper and lower extremity spasticity may be treated. Vigilance must be maintained for the
possibility of pump malfunction, as symptoms of baclofen withdrawal can be severe and potentially life-threatening. Patients who are ambulatory and
whose spasticity primarily affects their lower extremities may be candidates for an SDR. It is thought that inputs entering the spinal cord through the
dorsal roots have a net excitatory effect on the ventral motor roots, thus contributing to spasticity. The premise of an SDR is to intraoperatively
stimulate lumbosacral dorsal nerve rootlets and record responses from the anterior nerve roots and muscles. This allows for the identification of
those nerve rootlets that are relatively more involved in maintaining hypertonia, and such nerve rootlets are sectioned. SDR has been shown to
improve ambulation, but the procedure does not confer previously nonambulatory patients with the ability to walk.
PEDIATRIC TRAUMA AND BIRTH INJURIES
General Principles
Head injury and its management are discussed elsewhere, and treatment principles used in the management of adult trauma also apply to the
pediatric patient. Certain aspects of trauma that are unique to the pediatric population are highlighted here.
Head injuries are 30 times more common in children than spinal cord injuries, and they represent the most common cause of mortality and morbidity
in children. In infants and young children, the brain and head are disproportionately large compared to the trunk and torso, and the neck and
paraspinal musculature are incompletely developed. In children younger than 4 years of age, the skull is soft, is more prone to fracture, and provides
less protection to the brain. Most skull fractures can be treated conservatively, including some depressed skull fractures. Depressed skull fractures
that cause a significant cosmetic defect can be considered for elevation. In children less than 2 years of age, skull fractures associated with a tear in the
underlying dura may expand over time. These growing skull fractures are rare but may require operative closure of the dural defect when
encountered.
Nonaccidental Trauma
Nonaccidental head trauma is the leading cause of traumatic death and morbidity in children. Diagnosis of nonaccidental head trauma can be
challenging, and there may be few signs of external trauma even with significant neurologic injury. The child may present with lethargy, irritability,
poor feeding, apneic episodes, or seizures. Multiple skull fractures that are associated with underlying brain injury, bilateral chronic SDHs or SDHs of
varying ages, SAH, and retinal hemorrhages should raise the index of suspicion for child abuse. SDHs commonly occur along the bilateral convexities
or in the posterior interhemispheric fissure. MRI is best at evaluating SDHs of varying ages, as well as the extent of DAI that occurs with the acceleration-
deceleration and rotational forces in shaken baby syndrome. A workup should include imaging of the brain and possibly spine, a skeletal survey to
assess for long-bone or rib fractures, a funduscopic examination to check for retinal hemorrhages, and a thorough external exam to assess for
bruising. Death from nonaccidental trauma is most often due to refractory intracranial hypertension.
Spine Trauma
SCI is relatively rare in the pediatric population. The pediatric spine continues to develop throughout the first 2 decades of life. Ligamentous injury is
more common than bony injury, owing to ligamentous laxity, immature supporting musculature, and the developing bony joints of the spine. The
cervical spine is most commonly injured in children, and in children less than 9 years of age, a higher percentage of injuries occur in the upper cervical
spine.
Al-Holou WN, Yew AY, Boomsaad ZE, et al: Prevalence and natural history of arachnoid cysts in children. J Neurosurg Pediatr . 2010:5(6):578–585.
[PubMed: 20515330]
Klimo P, Rao G, Brockmeyer D: Pediatric arteriovenous malformations: a 15-year experience with an emphasis on residual and recurrent lesions.
Childs Nerv Syst. 2007;23(1):31–37. [PubMed: 17053936]
more common than bony injury, owing to ligamentous laxity, immature supporting musculature, and the developing bony joints of the spine. The
cervical spine is most commonly injured in children, and in children less than 9 years of age, a higher percentage of injuries occur in the upper cervical
spine. Access Provided by:
Al-Holou WN, Yew AY, Boomsaad ZE, et al: Prevalence and natural history of arachnoid cysts in children. J Neurosurg Pediatr . 2010:5(6):578–585.
[PubMed: 20515330]
Klimo P, Rao G, Brockmeyer D: Pediatric arteriovenous malformations: a 15-year experience with an emphasis on residual and recurrent lesions.
Childs Nerv Syst. 2007;23(1):31–37. [PubMed: 17053936]
Lew SM, Kothbauer KF: Tethered cord syndrome: an updated review. Pediatr Neurosurg. 2007;43:236–248. [PubMed: 17409793]
Nield LS, Brunner MD, Kamat D: The infant with a misshapen head. Clin Pediatr. 2007;46:292–298.
Ramaswamy V, Remke M, Bouffet E, et al: Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. Acta
Neuropathol. 2016;131(6):821–831. [PubMed: 27040285]
Shu HG, Sall WF, Maity A, et al: Childhood intracranial ependymomas. Twenty-year experience from a single institution. Cancer. 2007;110:432–441.
[PubMed: 17559078]
Tubbs RS, Lyerly MJ, Loukas M, et al: The pediatric Chiari I malformation: a review. Childs Nerv Syst. 2007;23(11):1239–1250. [PubMed: 17639419]
INTRACRANIAL ANEURYSMS AND ARTERIOVENOUS MALFORMATIONS

Pui Man Rosalind Lai, MD, and Timothy Smith, MD, PhD, MPH
INTRACRANIAL ANEURYSMS
KEY CONCEPTS
An intracranial aneurysm is an abnormal dilation or expansion of an artery commonly occurring at branch points in the circle of Willis.
Most aneurysms are asymptomatic, but if they rupture, they carry a significant rate of morbidity and mortality.
Treatment options include surgical clipping or endovascular coiling of the aneurysm to prevent future rupture.
General Consideration
Intracranial aneurysm, or cerebral aneurysm, is an abnormal dilation or expansion of an intracranial artery. Autopsy studies suggest these lesions are
relatively common, presenting in 1%-5% of the adult population. Aneurysms are classified into different types based on their morphology: saccular,
fusiform, and dissecting. Saccular aneurysms are the most common type of aneurysms and are commonly located at the branch points in the circle of
Willis. These aneurysms are thought to be sporadically acquired lesions, but rare familial forms have been described in the literature. Intracranial
aneurysms are associated with a number of heritable connective tissue disorders, including autosomal dominant polycystic kidney disease,
fibromuscular dysplasia, Marfan syndrome, and Ehlers-Danlos syndrome type IV.
The exact pathophysiology of intracranial aneurysms is still to be determined, but it is known that genetics as well as environmental factors, such as
hypertension and smoking, play a significant role in the progression of disease. Histologically, the aneurysmal wall has a decreased tunica media, the
middle muscular layer of the artery, resulting in structural defects and an outpouching at the arterial branch point.
Diagnosis & Clinical Findings
Most intracranial aneurysms remain asymptomatic and undetected, but if they rupture, they can present as devastating and life-threatening events.
The estimated incidence of an aneurysmal rupture in the United States is 1 case per 10,000 persons, with peak incidence in persons 55-60 years old,
and they occur more commonly in women. Typically, for less severe bleeds, patients often describe an acute onset of severe headache with nuchal
rigidity and lethargy, often known as “the worst headache of life.” The Hunt and Hess grading scale is commonly used to convey the severity of
symptoms and to risk-stratify patients (Table 38–6). Higher Hunt and Hess grade is associated with worse clinical outcome.
Table 38–6.
Hunt-Hess scale.
Most intracranial aneurysms remain asymptomatic and undetected, but if they rupture, they can present as devastatingAccess Provided by:
and life-threatening events.
The estimated incidence of an aneurysmal rupture in the United States is 1 case per 10,000 persons, with peak incidence in persons 55-60 years old,
and they occur more commonly in women. Typically, for less severe bleeds, patients often describe an acute onset of severe headache with nuchal
rigidity and lethargy, often known as “the worst headache of life.” The Hunt and Hess grading scale is commonly used to convey the severity of
symptoms and to risk-stratify patients (Table 38–6). Higher Hunt and Hess grade is associated with worse clinical outcome.
Table 38–6.
Hunt-Hess scale.
Grade Findings
I Mild headache or nuchal rigidity
II Severe headache, nuchal rigidity, possible cranial nerve deficit
III Lethargic, confused, mild focal deficit
IV Stuporous, moderate to severe hemiparesis, early decerebrate posturing
V Deep coma, decerebrate posturing, moribund
Reproduced with permission from Hunt WE, Hess RM: Surgical repair as related to time of intervention in the repair of intracranial aneurysms. J Neurosurg 1968
Jan;28(1):14-20.
Aneurysms that have hemorrhaged remain at risk for new rebleeding. The estimated rehemorrhage rate of a ruptured aneurysm is approximately 25%
within the first 2 weeks and 50% by 6 months. Because mortality of a rebleed approaches 80%, referral to a neurosurgeon for urgent treatment of a
ruptured aneurysm is critical for patient outcome.
Anatomically, the intracranial arteries course within the subarachnoid space. An aneurysmal rupture often results in hemorrhage in this space (SAH).
Noncontrast head CT has a high sensitivity for the detection of SAH, with a characteristic pattern of blood in the basal cisterns, the CSF spaces
surrounding the cortex, and the cerebellum.
An enlarging but unruptured intracranial aneurysm may also present as focal neurologic deficit by its mass effect. The presenting symptom is
dependent on the location of the aneurysm. The most common cranial nerve palsy due to mass effect from an aneurysm is a third nerve palsy, through
compression of the oculomotor nerve (CN III) by the posterior communicating artery or the posterior cerebral artery. Aneurysms larger than 2.5 cm are
called giant aneurysms and may lead to more pronounced symptoms including hemiparesis, obstructed hydrocephalus, seizures, and brainstem
compression.
With the increasing availability and advancement of neuroimaging, the detection of unruptured and asymptomatic cerebral aneurysms is increasing
(Figure 38–21). Although aneurysms that have ruptured or are causing focal neurologic deficit should be treated as emergencies, the decision for
treatment of incidental aneurysms is more complex and is dependent on many factors, including the patient’s age and comorbidities and aneurysm
location and morphology.
Figure 38–21.
An anterior view of left carotid artery cerebral angiogram demonstrating a large cerebral artery aneurysm (arrow) at the middle cerebral artery
bifurcation.
Figure 38–21.
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An anterior view of left carotid artery cerebral angiogram demonstrating a large cerebral artery aneurysm (arrow) at the middle cerebral artery
bifurcation.
Treatment
A. Initial Management
The determination of a patient’s airway, breathing, and circulation (ABCs) should be performed in patients with suspected ruptured intracranial
aneurysms. Patients with unstable or unprotected airways should be intubated and placed on mechanical ventilation. The maintenance of PCO2 values
between 28 and 32 mm Hg can help acutely lower ICP. Blood pressure should be maintained with intravenous medications as needed, with close
monitoring by placement of an arterial line. Basic laboratory values should be assessed including complete blood count, coagulation levels, sodium
level, blood urea nitrogen, and creatinine. An electrocardiogram should be performed to rule out neurogenic stress cardiomyopathy.
The demonstration of SAH on an initial head CT and no traumatic history should raise suspicion for an intracranial vascular lesion and should be
immediately followed by an assessment of the cerebral vasculature by CTA or conventional angiography (Figure 38–22). CSF diversion, typically
through an EVD, may be required if a patient presents with hydrocephalus or has concerns for elevated ICP.
Figure 38–22.
Classic appearance of a large subarachnoid hemorrhage. Notice the hemorrhage pattern fills the cerebrospinal fluid spaces at the base of the brain
and around the brainstem.
Figure 38–22.
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Classic appearance of a large subarachnoid hemorrhage. Notice the hemorrhage pattern fills the cerebrospinal fluid spaces at the base of the brain
and around the brainstem.
If an initial head CT did not demonstrate SAH but clinical suspicion is high, a lumbar puncture is often performed to assess for xanthochromia, the
yellow discoloration indicating the presence of bilirubin in CSF.
B. Surgical and Endovascular Treatment
The two major modalities for the treatment of an intracranial aneurysm are microsurgical clipping and endovascular coiling. Microsurgical clipping
involves an open craniotomy and the application of a surgical clip around the neck of the aneurysm to prevent rupture of the aneurysm. Endovascular
treatment uses conventional angiography and navigating a microcatheter to the aneurysmal defect to insert detachable coils into the aneurysm dome.
These coils promote thrombus formation to prevent blood entering the aneurysm. More recently, endovascular flow diversion has also been
introduced, which involves placing a device in the parent blood vessel to divert blood flow away from the aneurysm. The decision to secure the
cerebral aneurysm through microsurgical clipping or endovascular coiling is beyond the scope of this text. Factors for consideration include patient’s
age, overall medical condition, aneurysmal characteristics such as size, location, morphology, and rupture status of the aneurysm.
C. Postoperative Management
The management of patients remains particularly challenging, and often, patients require prolonged intensive care hospitalization due to concerns for
the development of delayed cerebral ischemia and permanent neurologic deficits, including hemiparesis, aphasia, and visual disturbances. This
phenomenon has historically been thought to be due to cerebral vasospasm, the constriction of cerebral blood vessels as a result of the SAH, resulting
in ischemia and infarct. The prevalence of vasospasm has been reported in up to 67% of patients presenting with SAH and typically appears between 3
and 14 days after the initial ictus, peaking at 7-10 days. However, only 20%-30% of patients with radiographic findings of vasospasm present with
clinical symptoms, and those who develop clinical deficits may not demonstrate radiographic vasospasm, suggesting that the development of
neurologic ischemia is not simply due to mechanical constriction of blood vessels. The development of neurologic deficits and infarcts, more recently
termed delayed ischemic neurologic deficits, is now thought to be more complex, including microthrombosis and the loss of cerebral autoregulation.
The management of cerebral vasospasm often involves a regimen referred to as triple H therapy: hypertension (by the use of vasopressors),
hemodilution, and hypervolemia (by the use of albumin or hypertonic solution) to increase cerebral perfusion. Patients are typically placed on
nimodipine, a calcium channel blocker that has been demonstrated to decrease overall mortality but does not affect vasospasm. For severe cerebral
vasospasm, cerebral angioplasty may be performed to inject calcium channel blockers directly into the affected blood vessels.
Outcome/Prognosis
The strongest predictors of outcome after an aneurysmal rupture are patient age, comorbidities, and Hunt and Hess grade. The overall mortality rate
of an aneurysmal rupture is 30%-50%, and 10%-20% of patients die before reaching a hospital. For patients who survive the initial insult, mortality
rates range from 10%-20%, with morbidity rates of 20%-40% secondary to initial aneurysmal bleed or delayed neurologic ischemia.
Summary
hemodilution, and hypervolemia (by the use of albumin or hypertonic solution) to increase cerebral perfusion. Patients are typically placed on
nimodipine, a calcium channel blocker that has been demonstrated to decrease overall mortality but does not affect vasospasm. For severe cerebral
vasospasm, cerebral angioplasty may be performed to inject calcium channel blockers directly into the affected blood vessels.
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Outcome/Prognosis
The strongest predictors of outcome after an aneurysmal rupture are patient age, comorbidities, and Hunt and Hess grade. The overall mortality rate
of an aneurysmal rupture is 30%-50%, and 10%-20% of patients die before reaching a hospital. For patients who survive the initial insult, mortality
rates range from 10%-20%, with morbidity rates of 20%-40% secondary to initial aneurysmal bleed or delayed neurologic ischemia.
Summary
Intracranial aneurysms carry significant morbidity and mortality if they rupture. Because aneurysms have an increased rate of rebleeding after an
initial hemorrhage and are often fatal, ruptured aneurysms should be treated urgently by microsurgical clipping or endovascular coiling. Patients
remain at risk for delayed neurologic deficits and often require prolonged hospitalization after a ruptured aneurysm.
ARTERIOVENOUS MALFORMATIONS
KEY CONCEPTS
An AVM is a congenital, abnormal collection of arteries and veins that may cause intraparenchymal hemorrhage when ruptured.
Treatment may include surgery and radiosurgery to prevent hemorrhage or observation in some cases.
AVMs are tangles of congenital blood vessels with no intervening capillary bed, forming an abnormal fistula between arteries and veins (Figure 38–23).
The majority of AVMs are found in the supratentorial space, but they can also be present in the brainstem and in the spine. Autopsy data suggest that
AVMs occur in 1%-4% of the population, estimated to be 10-18 per 100,000 adults. Intracranial AVMs are seen in sporadic cases and rarely in some
genetic syndromes, such as Osler-Weber-Rendu syndrome.
Figure 38–23.
Cerebral angiogram demonstrating a frontal arteriovenous malformation filling from the left anterior cerebral artery.
Hemorrhage is the most common presentation for an AVM, occurring in over 50% of patients. Other common presentations include seizures,
headache, and focal neurologic symptoms from mass effect. Initial CT findings most often present as an intraparenchymal hemorrhage, and
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Hemorrhage is the most common presentation for an AVM, occurring in over 50% of patients. Other common presentations include seizures,
headache, and focal neurologic symptoms from mass effect. Initial CT findings most often present as an intraparenchymal hemorrhage, and
subsequent angiographic modalities to assess for the cerebral vasculature are critical to confirm the diagnosis of an AVM.
For unruptured AVMs, the estimated rate of hemorrhage is approximately 2%-4% per year. Rebleeding rate in the first year after hemorrhage is 6%.
Treatment
A. Initial Management
The initial evaluation of AVMs depends on the patient’s presentation. For patients with hemorrhage from AVM, the fundamentals of emergency care
(ABCs) and complete neurologic evaluation are critical. Blood pressure management and the placement of an arterial line may be necessary.
Antiepileptics should be administered for seizure prophylaxis.
After the initial confirmation of the diagnosis on CTA, the standard approach often includes a conventional angiography to assess radiographic
features of the AVM. The most well-known classification for AVM is the Spetzler-Martin grading scale, which uses AVM size, venous drainage (superficial
or deep), and eloquence of surrounding brain tissue, and is used to assess surgical morbidity and mortality (Table 38–7).
Table 38–7.
Spetzler-Martin arteriovenous malformation (AVM) grading scale.
Graded Feature Points Assigned
Size of AVM 1
< 3 cm 2
3-6 cm 3
> 6 cm
Eloquence1 of adjacent brain 0
Noneloquent 1
Eloquent
Venous drainage 0
Superficial 1
Deep
1Eloquent areas include visual, language, and sensorimotor cortex; the thalamus and hypothalamus; the internal capsule; the brainstem; the cerebellar peduncles;
and the deep cerebellar nuclei.
B. Treatment for AVM
The treatment modalities for AVM include microsurgical resection, endovascular embolization, stereotactic radiosurgery, or a combination of the
above approaches. The decision on which approach to secure the AVM is beyond the scope of this chapter, but factors considered include AVM
location and grade and patient preference.
Microsurgical resection of the AVM is the gold standard, which involves an open craniotomy and complete obliteration of the AVM. Most
neurosurgeons agree that Spetzler-Martin grade I-III AVMs that are easily accessible should be surgically resected due to low complication rates.
However, grade IV and V lesions carry a much higher risk for morbidity and mortality, up to 30%, and patients should be carefully selected before
proceeding with treatment. Endovascular embolization of blood vessels may sometimes assist in reducing the size of AVM prior to surgical resection.
Stereotactic radiosurgery (SRS) may be an alternative for AVMs located in deep locations or patients who are poor surgical candidates. SRS is most
ideal for AVMs less than 3 cm. The major disadvantage of this method is the 2-3 years it takes for the AVM to involute, and patients remain at risk of
hemorrhage during this time.
above approaches. The decision on which approach to secure the AVM is beyond the scope of this chapter, but factors considered include AVM
location and grade and patient preference.
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Microsurgical resection of the AVM is the gold standard, which involves an open craniotomy and complete obliteration of the AVM. Most
neurosurgeons agree that Spetzler-Martin grade I-III AVMs that are easily accessible should be surgically resected due to low complication rates.
However, grade IV and V lesions carry a much higher risk for morbidity and mortality, up to 30%, and patients should be carefully selected before
proceeding with treatment. Endovascular embolization of blood vessels may sometimes assist in reducing the size of AVM prior to surgical resection.
Stereotactic radiosurgery (SRS) may be an alternative for AVMs located in deep locations or patients who are poor surgical candidates. SRS is most
ideal for AVMs less than 3 cm. The major disadvantage of this method is the 2-3 years it takes for the AVM to involute, and patients remain at risk of
hemorrhage during this time.
Due to the high risk of complications with higher grade AVMs, some surgeons advocate conservative treatment for these AVMS, although opinions on
the treatment for AVMs can vary widely among neurosurgeons and neurointerventionalists. AVMs are complex vascular lesions and should be treated
by neurosurgeons in high-volume centers with significant experience.
C. Postoperative Management
Patients with hemorrhage from AVMs should be observed in the ICU with close observation of their neurologic status. After the obliteration of a large
AVM, a phenomenon known as normal perfusion pressure breakthrough may occur. As the pathologic shunting of blood through the AVM nidus is
removed, there is an increase in blood flow to the surrounding blood vessels and brain, which may result in hemorrhage. For these reasons, blood
pressure monitoring is critical in postoperative management of AVM.
Spinal Arteriovenous Malformations
Spinal AVMs are rare vascular entities and are commonly classified into four types: (1) dural arteriovenous fistulas; (2) glomus AVMs; (3) juvenile
intradural AVMs; and (4) intradural, extramedullary arteriovenous fistulas. Type 1 AVMs are the most common spinal vascular malformations and
commonly occur near the thoracolumbar junction, with one arterial feeding vessel that connects to an intradural arterialized vein. Symptoms from
spinal AVMs are commonly due to hemorrhage or venous congestion, resulting in acute or subacute lower extremity neurologic deficit, gait difficulties,
myelopathy, or loss of bladder or bowel control. Initial diagnostic evaluation should include spinal MRI/MR angiography, and often, spinal angiography
is performed to evaluate the vascular lesion.
Summary
Intracranial aneurysms and AVMs are rare vascular entities that are often asymptomatic but that can be devastating after a hemorrhage. The morbidity
and mortality associated with intracranial hemorrhage are high. As such, treatment is often warranted, including open neurovascular or
neuroendovascular surgery, and these patients should be referred to and cared for by experienced teams of neurosurgeons, interventionalists, and
neurocritical care specialists.
Solomon RA, et al: Arteriovenous malformations of the brain. N Engl J Med . 2017;376:1859–1866. [PubMed: 28489992]
SURGICAL MANAGEMENT OF MEDICALLY INTRACTABLE REFRACTORY EPILEPSY

Pui Man Rosalind Lai, MD, and Timothy Smith, MD, PhD, MPH
KEY CONCEPTS
Epilepsy is a syndrome of recurrent seizures and affects 1% of the population.
Surgery is often reserved for structural lesions causing seizures or patients who have failed maximal medical therapy.
INTRODUCTION
Epilepsy is the second most commonly reported neurologic condition worldwide and affects 1% of the world population. Epilepsy is a syndrome of
recurrent seizures, with an onset of seizure due to abnormal neuronal synchronization and propagation to surrounding brain areas. The first-line
treatment for epilepsy is medical therapy, which involves pharmacotherapy and lifestyle changes, but those who fail medical treatment may be
candidates for surgical intervention.
Seizure Classification
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INTRODUCTION
Epilepsy is the second most commonly reported neurologic condition worldwide and affects 1% of the world population. Epilepsy is a syndrome of
recurrent seizures, with an onset of seizure due to abnormal neuronal synchronization and propagation to surrounding brain areas. The first-line
treatment for epilepsy is medical therapy, which involves pharmacotherapy and lifestyle changes, but those who fail medical treatment may be
candidates for surgical intervention.
Seizure Classification
A seizure is an abnormal cerebral neuronal discharge that results in changes in sensation, motor, behavior, or consciousness. Seizures can be
classified by their associations with alteration in consciousness and their etiology. Partial seizures involve one hemisphere and can further be
categorized as simple partial, defined as those that occur with no impairment of consciousness, or complex partial, resulting in a loss of awareness.
Generalized seizures involve synchronous activity in the bilateral hemisphere, and generalized tonic-clonic seizure (grand mal seizure) is defined as a
specific type of generalized seizure that evolves from tonic to clonic motor activity.
DIAGNOSIS
Several modalities are used in the diagnosis of epilepsy, which include clinical examination, neurophysiology, neuroimaging, and neuropsychologic
assessments, and all are critical for consideration for patients who may be candidates for surgery.
Clinical Examination & Laboratory Assessment
The semiology, or clinical symptomatology, is a critical component of the diagnosis of epilepsy. The symptoms during the time of seizure may provide
localizing clues to the region of onset. For example, seizures starting with motor twitching of the upper extremity likely originate from the vicinity of the
primary motor cortex. Serum studies, such as fasting blood glucose, serum electrolyte panel, complete blood count, and toxicology, should also be
undertaken to rule out potentially reversible causes of seizures.
Electrophysiology
The standard modality for recording neuronal activity is the scalp EEG. EEG recordings may be obtained between and during seizures to assess for
semiology and seizure focus. In patients whose seizure localization cannot be proven by scalp EEG, intracranial EEG electrodes may be placed. These
electrodes can either be placed on the surface of the brain for cortical activity (subdural electrodes) or within the brain for deeper structures like the
hippocampus (depth electrodes).
Imaging studies
High-resolution MRI of the brain is the gold standard for the evaluation of structural lesions, such as tumors, vascular malformations, dysplastic cortex,
and mesial temporal sclerosis (Figure 38–24). For patients who have normal MRIs or those with uncertainty about the site of seizure origin, nuclear
medicine may be useful to identify focus area. PET and SPECT may be additionally performed to measure metabolic activity and regional blood flow
patterns of the brain. SPECT studies performed at the beginning of a seizure may demonstrate increased blood flow in areas involved in seizure onset.
PET studies can be performed during or between seizures and, when performed between seizures, demonstrate hypometabolism with the
epileptogenic foci.
Figure 38–24.
Coronal magnetic resonance imaging of the brain demonstrating right mesial temporal sclerosis with associated atrophy of the right hippocampus and
prominence of the temporal horn of the right lateral ventricle.
Figure 38–24.
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Coronal magnetic resonance imaging of the brain demonstrating right mesial temporal sclerosis with associated atrophy of the right hippocampus and
prominence of the temporal horn of the right lateral ventricle.
Neuropsychological Evaluation
All individuals who are candidate for surgery for the treatment of epilepsy should undergo a neuropsychological evaluation, which is conducted by a
neuropsychologist to assess a person’s cognitive abilities that may have been affected by epilepsy. The testing includes concentration, memory, and a
range of verbal and visual abilities.
GOALS OF SURGERY
Surgery is the primary treatment for patients with structural lesions such as tumors and vascular malformations. In these patients, the goal of surgery
is curative through complete resection of the affected lesion or cortex, if safe to remove.
Individuals who have failed medical therapy may also be considered for epilepsy surgery. The likelihood of attaining seizure freedom with
pharmacotherapies after two drug failures has been shown to be less than 5%, and therefore, a consensus definition of medical failure is currently
defined as failure of two or more antiepileptic drugs to reduce seizures to a clinically meaningful level. For these individuals, a palliative surgical
approach is often employed for the reduction in seizure frequency and severity.
SURGICAL PROCEDURES FOR EPILEPSY
Surgical Resection or Disconnection
For patients who have identifiable epileptic foci due to a structural lesion, surgical resection is the optimal treatment. These patients undergo a
craniotomy, and the brain tissue containing the epileptic foci is resected, but this operation is limited to noneloquent brain areas. The most common
cause of drug-resistant epilepsy is mesial temporal sclerosis, and these patients are candidates for temporal lobectomy, which involves resection of
the amygdala, parahippocampal gyrus, and hippocampus. Observational studies have found long-term seizure-free outcomes to be up to 60%-80%,
but potential complications for this procedure include short-term memory problems and minor visual field deficits. An alternative similar technique is
the selective amygdalohippocampectomy, which involves the resection of the amygdala and hippocampus only. Comparisons of the standard anterior
temporal lobectomy and selective amygdalohippocampectomy have found similar seizure-freedom rates.
In patients without a detectable lesion by MRI or a resectable seizure focus, disconnection procedures may be performed, with the goal of interrupting
the synchronous firing of neurons. Anatomic hemispherectomy is the complete removal of half of the brain, used in patients with diffuse unilateral
hemispheric seizures. More recently, hemispherectomy has been replaced by functional hemispherectomy, which involves the disconnection of the
corpus callosum and various interhemispheric commissures. Functional hemispherectomy is more commonly performed in the pediatricPage
Chapter 38: Neurosurgery, Timothy R. Smith 72 / 93
population,
such as in patients with Sturge-Weber syndrome, Rasmussen encephalitis, and porencephaly. Corpus callosotomy is another palliative disconnective
procedure for patients with diffuse epileptic foci involving bilateral hemispheres. This procedure involves resection of the anterior two-thirds of the
but potential complications for this procedure include short-term memory problems and minor visual field deficits. An alternative similar technique is
the selective amygdalohippocampectomy, which involves the resection of the amygdala and hippocampus only. Comparisons of the standard anterior
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temporal lobectomy and selective amygdalohippocampectomy have found similar seizure-freedom rates.
In patients without a detectable lesion by MRI or a resectable seizure focus, disconnection procedures may be performed, with the goal of interrupting
the synchronous firing of neurons. Anatomic hemispherectomy is the complete removal of half of the brain, used in patients with diffuse unilateral
hemispheric seizures. More recently, hemispherectomy has been replaced by functional hemispherectomy, which involves the disconnection of the
corpus callosum and various interhemispheric commissures. Functional hemispherectomy is more commonly performed in the pediatric population,
such as in patients with Sturge-Weber syndrome, Rasmussen encephalitis, and porencephaly. Corpus callosotomy is another palliative disconnective
procedure for patients with diffuse epileptic foci involving bilateral hemispheres. This procedure involves resection of the anterior two-thirds of the
corpus callosum, with the greatest efficacy for drop attacks, also knowns as sudden atonic seizures (up to 88%).
Ablative Procedures
Ablation of epileptogenic brain tissue using highly focused radiation (SRS) has been used to target a wide variety of brain lesions such as tumors and
vascular lesions. The advantage is its less invasive nature and precise targeting of seizure-producing foci, but benefits are delayed (usually starting 12
months after procedure), and there is risk of brain edema in neighboring tissue.
Therapeutic Devices
Electrical stimulation of peripheral nerves, particularly through vagus nerve stimulation, has been used for patients whose disease is not safely
amenable to resection. The exact mechanism is unknown but has been postulated to be due to innervation of the nucleus solitarius that sends fibers
to hemispheric regions involved in seizure onset. The stimulator electrode is wrapped around the vagus nerve in the neck and connected to a
generator implanted on the anterior chest wall. Randomized trials have demonstrated a reduction of seizure frequency to 30%, with greater response
rates in the pediatric population.
A more recent advancement includes the placement of an electric stimulator implant into the brain that detects seizure onset and responds by
delivering pulsed stimulation to the epileptic focus. The preliminary trials are promising, with up to 50%-70% reduction in seizures. The development
of therapeutic devices for refractory epilepsy remains an ongoing research area.
SUMMARY
Epilepsy surgery may be considered for patients with disabling epilepsy who have failed medical therapy. The mapping of epileptogenic origin and
careful selection of patients and correct procedures require a multidisciplinary team of epileptologists, neurosurgeons, radiologists, and
neuropsychiatrists to best maximize efficacy, with the goal to ultimately improve quality of life for these patients.
Immonen A, Jutila L, Muraja-Murro A, et al: Long-term epilepsy surgery outcomes in patients with MRI-negative temporal lobe epilepsy. Epilepsia.
2010;51(11):2260–2269. [PubMed: 21175607]
Schachter SC, Guttag J, Schiff SJ, et al: Advances in the application of technology to epilepsy: the CIMIT/NIO Epilepsy Innovation Summit. Epilepsy
Behav . 2009;16:3–46. [PubMed: 19780225]
SURGICAL MANAGEMENT OF PAIN

The subjective, emotional, and physical components of pain make its management complex and often require interdisciplinary collaboration. Surgical
intervention for pain is uncommon and should be reserved for patients who are refractory to other therapies.
Pain may be classified as nociceptive or neuropathic. Nociceptive pain results from tissue injury and is often described as a constant aching or
throbbing, with responsiveness to opiate medications. Neuropathic pain results from nerve damage and is typically described as electrical, burning, or
intense numbness, often with allodynia as well. Neuropathic pain responds poorly to opiate medications. Pain surgeons should be familiar with these
concepts to assess medical intractability.
Surgical management of pain typically involves either the interruption or modulation of afferent pain pathways. Ablative surgical techniques involve
the destruction of neural tissue, whereas nonablative procedures involve functional modulation of pain transduction pathways.
ABLATIVE PROCEDURES
Neurosurgical procedures to physically interrupt pain signaling have been directed toward the nerves (neurectomy), spinal roots (rhizotomy), dorsal
root ganglia (ganglionectomy), dorsal root entry zone (DREZ lesioning), spinal cord (cordotomy, myelotomy), and cerebral cortex (thalamotomy,
throbbing, with responsiveness to opiate medications. Neuropathic pain results from nerve damage and is typically described as electrical, burning, or
intense numbness, often with allodynia as well. Neuropathic pain responds poorly to opiate medications. Pain surgeons should be familiar with these
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concepts to assess medical intractability.
Surgical management of pain typically involves either the interruption or modulation of afferent pain pathways. Ablative surgical techniques involve
the destruction of neural tissue, whereas nonablative procedures involve functional modulation of pain transduction pathways.
ABLATIVE PROCEDURES
Neurosurgical procedures to physically interrupt pain signaling have been directed toward the nerves (neurectomy), spinal roots (rhizotomy), dorsal
root ganglia (ganglionectomy), dorsal root entry zone (DREZ lesioning), spinal cord (cordotomy, myelotomy), and cerebral cortex (thalamotomy,
cingulotomy). Ablative surgery for pain is most often used in the treatment of cancer-related, nociceptive pain, because long-term analgesia is less
commonly observed for these procedures.
Neurectomy
Neurectomy involves cutting an injured nerve or the nerve to a specific painful region and can have use in pain following peripheral nerve injury such
as from limb amputation and/or neuroma formation. Denervation of joints or distal sensory nerves and neuroma surgery are examples of peripheral
neurectomy. The utility of neurectomy is limited, however, because it is rare for the pain to arise from an isolated sensory nerve. More commonly, pain
distributions follow a mixed motor-sensory nerve distribution, and the nerve cannot be sectioned without inducing a disabling motor deficit.
Moreover, as neurostimulation has gained in popularity, neurectomy has lost ground because it is irreversible and seen as more invasive.
Dorsal Rhizotomy and Ganglionectomy
Dorsal rhizotomy and ganglionectomy target the destruction of the dorsal sensory rootlets or ganglia, respectively, and can produce denervation of a
specific dermatome. The procedures are used most commonly for regional cancer-related pain such as thoracic or abdominal wall pain, perineal pain
in those with impaired pelvic visceral function, or limb pain in those with preexisting loss of function. Unfortunately, the procedures do not typically
have durable results in non–cancer-related pain conditions, although some success in occipital neuralgia, specifically, has been reported. These
procedures are rarely used in the treatment of extremity pain because of functional impairment resulting from the loss of proprioception.
Ganglionectomy is thought to be slightly more efficacious than rhizotomy due to the ability to catch any afferent fibers that enter through the ventral
root, whose bodies nonetheless reside in the dorsal root ganglion.
Dorsal Root Entry Zone Lesioning
Dorsal root entry zone surgery targets the superficial dorsal horn region of the spinal cord, where sensory fibers enter. Levels including and flanking
the region of interest, as defined by imaging studies or pain distribution, are typically ablated. A knife or radiofrequency heating is used to make the
lesion.
Dorsal root entry zone surgery is most effective in the treatment of regionally specific neuropathic pain following nerve root avulsion and “at-level” or
“boundary-level” SCI pain. Risks of surgery include injury to descending motor pathways and decreased sensory function in the territory of the ablated
region. In carefully selected patients, rates of successful pain control range from 60% to 80%.
Cordotomy & Myelotomy
Cordotomy is a spinal procedure to interrupt pain transmission along the lateral spinothalamic tract. Cordotomy is most commonly performed in
patients with intractable, unilateral, nociceptive cancer pain at the level of the chest or below. The procedure may be performed either with a knife or
with radiofrequency heating and can be performed in an open or CT-guided fashion. Risks of surgery include lower extremity weakness, ataxia, and
respiratory or urinary dysfunction. These risks are significantly increased when cordotomy is performed bilaterally. As the use of intrathecal analgesics
has increased, the popularity of cordotomy has correspondingly declined.
A similar procedure, the midline myelotomy, involves the sectioning of a different spinal cord tract, the mesial dorsal columns, at a single spinal level. It
can be useful in treating midline, bilateral, or visceral pain without the associated risks of bilateral cordotomies. The procedure typically preserves
dorsal column and spinothalamic signal transmission. Risks include transient lower extremity paresthesias and weakness.
Rates of successful pain control with cordotomy and myelotomy are initially high (60%-90%) but decline over time (40% at 2 years).
Thalamotomy & Cingulotomy
Thalamotomy involves the surgical lesioning of the afferent pain pathway inside the thalamus using stereotactic-guided radiofrequency, radiosurgery,
or focused ultrasound ablation. It is used in either cancer- or non–cancer-related pain but is more appropriate for diffuse, widespread, nociceptive
pain as opposed to more regionally focused pain. Results are not as favorable as well-selected candidates for other modalities, with 30%-50% of
patients achieving acceptable long-term pain relief.
dorsal column and spinothalamic signal transmission. Risks include transient lower extremity paresthesias and weakness.
Rates of successful pain control with cordotomy and myelotomy are initially high (60%-90%) but decline over time (40%Access Provided by:
at 2 years).
Thalamotomy & Cingulotomy
Thalamotomy involves the surgical lesioning of the afferent pain pathway inside the thalamus using stereotactic-guided radiofrequency, radiosurgery,
or focused ultrasound ablation. It is used in either cancer- or non–cancer-related pain but is more appropriate for diffuse, widespread, nociceptive
pain as opposed to more regionally focused pain. Results are not as favorable as well-selected candidates for other modalities, with 30%-50% of
patients achieving acceptable long-term pain relief.
Unlike procedures directed along pathways of pain neurotransmission, cingulotomy is directed toward alteration of the experience of pain. Because
the cingulate gyrus is part of the limbic system, radiofrequency ablation of the anterior cingulate gyrus reduces the affective, unpleasant aspects of
pain, particularly in patients with obsessive, affective, and/or psychiatric components to their pain. Cingulotomy is performed in relatively few centers
and only in carefully selected patients. Following cingulotomy for intractable, cancer-related pain, over 50% of patients have been reported to have
moderate to complete pain relief.
PROCEDURES TO MODULATE AFFERENT PAIN PATHWAYS
Intrathecal Analgesic Delivery Pumps
When compared to oral narcotics, intrathecal administration of morphine provides more potent analgesia with reduced side effects, such as nausea,
constipation, and sedation. To benefit from intrathecal delivery, patients should have significant reduction in pain level with oral opiates, limited by
intolerable side effects.
Analgesics such as morphine are delivered through a catheter placed into the CSF of the spinal canal. The catheter tubing is then connected to an
external or surgically implanted pump. External pumps are used in patients with cancer-related pain and an expected survival of less than 3 months.
Principal complications of intrathecal drug delivery include the side effects of the medication, mechanical failure of the system, and infection.
Intrathecal drug delivery may be effective in either nociceptive or neuropathic pain syndromes. However, as with oral opiate administration, long-term
tolerance to medications can develop. Hence, these devices are most beneficial for patients with cancer-related pain syndromes and limited life
expectancy.
Peripheral Nerve & Spinal Cord Stimulation
Peripheral nerve and spinal cord stimulators deliver regular pulses of electricity to injured nerves or the dorsal columns of the spinal cord,
respectively. The mechanism of action by which stimulation works remains unclear but is thought to possibly involve preferential stimulation of large
afferent fibers, somehow blocking the pain signals from smaller fibers, as well as possible stimulation of secondary neurohormonal changes.
Although both neurostimulation techniques have been tried in a variety of chronic pain conditions, their use in a few specific indications has met with
the most success. Peripheral nerve stimulation is most effective in neuropathic peripheral nerve syndromes that fit well into a known nerve
distribution, such as occipital neuralgia. Spinal cord stimulation has been employed successfully in patients with failed back surgery syndrome,
ischemic limb pain, and complex regional pain syndrome.
Patient candidates typically undergo an initial trial with a temporary electrode applied to the spinal cord or nerve. After the trial period, permanent
placement can be performed if benefit is demonstrated, as evidenced by pain reduction and improvement to go about daily activities. Complications of
the therapy include migration or fracture of the simulator leads and infection, and although revisions are common, devastating complications that
significantly impact morbidity or mortality are rare.
Deep Brain Stimulation
Deep brain stimulation involves the precise surgical placement of electrodes into the deep nuclei of the brain. Common targets of deep brain
stimulation for pain include the thalamus, which is the sensory relay of the brain, and the periaqueductal gray region, which results in the upregulation
of endogenous opiates. Once electrodes are implanted, trial stimulation is performed, with success characterized by the experience of pain relief,
tolerable off-target sensations, and the absence of an analgesic effect during sham stimulation. Following the trial, a pulse generator is connected to
the wires and implanted in the chest. Long-term results in patients with a successful trial are highly variable.
Motor Cortex Stimulation
Electrical stimulation of the region of the motor cortex results in analgesia in neuropathic pain syndromes such as hemibody poststroke pain and
trigeminal deafferentation pain. The mechanism of motor cortex stimulation remains unknown.
Page 75 / 93
The surgical procedure involves placement of a stimulating electrode under the skull but over the dura in the region of the motor cortex. Patients
undergo a trial of stimulation, and following a successful trial, the electrodes are connected to an implantable pulse generator in the chest. As the
of endogenous opiates. Once electrodes are implanted, trial stimulation is performed, with success characterized by the experience of pain relief,
tolerable off-target sensations, and the absence of an analgesic effect during sham stimulation. Following the trial, a pulse generator is connected to
the wires and implanted in the chest. Long-term results in patients with a successful trial are highly variable. Access Provided by:
Motor Cortex Stimulation
Electrical stimulation of the region of the motor cortex results in analgesia in neuropathic pain syndromes such as hemibody poststroke pain and
trigeminal deafferentation pain. The mechanism of motor cortex stimulation remains unknown.
The surgical procedure involves placement of a stimulating electrode under the skull but over the dura in the region of the motor cortex. Patients
undergo a trial of stimulation, and following a successful trial, the electrodes are connected to an implantable pulse generator in the chest. As the
neuroanatomic representation of the leg lies further away, in between the two cerebral hemispheres, motor cortex stimulation is typically restricted to
those with arm or face pain.
SURGICAL MANAGEMENT OF MOVEMENT DISORDERS

PARKINSON DISEASE
Pathophysiology
Parkinson disease (PD) is the second most common progressive neurodegenerative disease, affecting 2%-3% of patients over 65 years of age. PD is the
most common and well-characterized basal ganglia disorder with both motor and nonmotor features and is characterized by loss of dopaminergic
neurons in the substantia nigra and accumulation of intracellular protein (alpha-synuclein) known as Lewy bodies. The net loss of dopaminergic input
in the substantia nigra causes opposing effects in the motor striatum, which leads to increased activity in the indirect pathway through net
disinhibition of the subthalamic nucleus (STN) and globus pallidus interna (GPi). As such, surgical approaches generally target either the STN or GPi.
Clinical Features
PD can display both motor and nonmotor symptoms, with the former including tremor, rigidity, akinesia/bradykinesia, postural instability, shuffling
gait, masked facies, and micrographia. Non-motor symptoms include cognitive impairment, apathy, fatigue, depression, sleep disorders, and
dysautonomia. PD is clinically defined by the presence of bradykinesia and at least one more cardinal feature and supporting/exclusion criteria. A
resting tremor characterized as a “pill rolling” tremor is the most common feature and is seen most prominently in the distal extremities. The clinical
history and presentation are key to distinguish PD from other parkinsonian syndromes that may be similar but require distinct treatment including
multiple system atrophy, corticobasal degeneration, progressive supranuclear palsy, and dementia with Lewy bodies.
Treatment
PD patients first undergo medical treatment for symptomatic control. Mainstay of treatment involves dopaminergic targets given the loss of
dopaminergic neurons in the substantia nigra. Levodopa (L-DOPA) is a precursor of dopamine and is the gold standard for PD. It is usually given in
combination with carbidopa (an inhibitor of dopamine metabolisms in the bloodstream) to increase its efficiency. Over a period of 5-10 years, patients
will naturally develop resistance to L-DOPA with motor response oscillations such as “on-off” fluctuations and drug-induced dyskinesias (involuntary
writhing movements of face and extremities at peak dopamine levels). Other medications used in the treatment of PD are monoamine oxidase
inhibitors (selegiline) and dopamine agonists (ropinirole).
Once patients start developing medical resistance, they should undergo surgical evaluation. Currently, the preferred surgical treatment options for PD
involve pallidotomy or deep brain stimulation (DBS) of the STN or GPi. The ideal DBS patient is generally a healthy patient without psychiatric disorders
who is L-DOPA responsive and has disabling idiopathic PD symptoms. In DBS surgery, deep subcortical electrodes are placed into the STN or GPi using
stereotactic techniques, with or without intraoperative electrophysiologic monitoring and with or without intraoperative imaging through small burr
holes. Stereotactic techniques use known atlas-based distances for each different nuclei (STN, GPi) in relation to the midcommissural point, anterior
commissure, and posterior commissure. Electrodes are connected to an implantable pulse generator located in an infraclavicular location. STN and
GPi DBS demonstrate comparable efficacy in addressing the cardinal symptoms of PD (bradykinesia, gait, rigidity, tremor) and reducing “on-off”
fluctuations. STN is a preferred target based on reduction of medication requirements (> 50% at 12 months) requiring lower stimulation voltage but
with more significant cognitive and behavioral side effects. The main complications with DBS include hemorrhage (2%), infection within 6 months
(4%), and hardware malfunction (8%). Pallidotomy, or ablation of the GPi, is a surgical option used preferably in patients with asymmetric symptoms
and those unable to comply with DBS follow-up and management. Unilateral pallidotomy has similar efficacy as unilateral DBS with the main
disadvantage being that of irreversible damage; otherwise, pallidotomy uses the same localization techniques as DBS, with lesioning performed using
radiofrequency ablation.
ESSENTIAL TREMOR
GPi DBS demonstrate comparable efficacy in addressing the cardinal symptoms of PD (bradykinesia, gait, rigidity, tremor) and reducing “on-off”
fluctuations. STN is a preferred target based on reduction of medication requirements (> 50% at 12 months) requiring lower stimulation voltage but
with more significant cognitive and behavioral side effects. The main complications with DBS include hemorrhage (2%),Access Provided by:
infection within 6 months
(4%), and hardware malfunction (8%). Pallidotomy, or ablation of the GPi, is a surgical option used preferably in patients with asymmetric symptoms
and those unable to comply with DBS follow-up and management. Unilateral pallidotomy has similar efficacy as unilateral DBS with the main
disadvantage being that of irreversible damage; otherwise, pallidotomy uses the same localization techniques as DBS, with lesioning performed using
radiofrequency ablation.
ESSENTIAL TREMOR
Pathophysiology
Essential tremor (ET) is the most common movement disorder characterized by benign and primary postural or intention tremor with usual age of
onset > 70 years of age, a slight female preponderance, and prevalence of 0.4%-6%. There is no consensus regarding the pathophysiologic basis of ET,
but the current working understanding suggests a neuronal loop involving the cerebellothalamocortical fibers and the ventral intermediate nucleus
(VIM) of the thalamus. The latter contains tremor cells that are posited as tremorigenic pacemakers involved in the tremor circuitry connecting
cerebellum and cortical motor pathways.
Clinical Features
ET is characterized by a postural and/or kinetic tremor with frequencies of 4-12 Hz that can be exacerbated by anxiety or improved with alcohol. The
intention tremor of ET should be carefully distinguished from physiologic tremor and other disease-associated tremors (eg, resting tremor with a
stationary limb seen with PD) or secondary tremors (eg, multiple sclerosis, stroke). Criteria for the diagnosis of ET include bilateral postural tremor
with duration greater than 5 years with or without an intentional component of forearms and arms and exclusion of other abnormal signs (eg, causes
increasing physiologic tremor, use of drugs inducing withdrawal, trauma, or stepwise deterioration).
Treatment
Similar to PD, ET patients have both medical and surgical options. First-line treatment for ET, especially when the disease impacts the activities of daily
living, are β-blockers, (eg, propranolol) and anticonvulsants (eg, primidone), with tremor reduction of up to 60% in 50% of patients. Second-line
agents include benzodiazepines, gabapentin, theophylline, calcium channel blockers, and botulinum toxin A. When medical therapy is insufficient,
patients should undergo surgical evaluation.
Patients with medically refractory ET notable for distal extremity tremor benefit the most from surgical intervention, with two main surgical options:
targeting of the VIM via DBS or thalamotomy using radiofrequency ablation, SRS, or focused ultrasound. DBS of the VIM can be unilateral or bilateral,
with success rates of > 70% and studies noting fewer adverse effects than with thalamotomy. Targeting techniques for VIM DBS in ET are similar to
those used for PD, with use of different coordinates for the VIM (unlike the GPi or STN for PD). Thalamotomy can be performed by means of the more
invasive radiofrequency approach, which, like pallidotomy in PD, is irreversible with reduction of contralateral tremor in > 85% of patients.
Thalamotomy is usually reserved for unilateral cases given the high rate (> 50%) of cognitive and bulbar defects with bilateral approaches or for
patients who are unable to tolerate or undergo long-term management with DBS. Two noninvasive surgical options for thalamotomy are focused
ultrasound and SRS. In focused ultrasound, patients undergo real-time MRI monitoring to target the VIM and undergo highly focused sonication with
acoustic energy to ablate tissue to temperatures of 55-60°C. A phase III, randomized controlled trial demonstrated > 50% improvement in tremor
scores. SRS has similar clinical outcomes to the aforementioned approaches but with a delayed clinical benefit and inability for intraoperative clinical
monitoring.
DYSTONIA
Pathophysiology
Dystonia is a hyperkinetic heterogeneous movement disorder characterized by sustained muscle contractions that generate repetitive movements,
abnormal postures, and/or twisting movements. Primary dystonias have no clearly defined etiology (eg, stroke, trauma) and demonstrate a bimodal
distribution at age 9 years (with appendicular symptoms) and 45 years (with axial symptoms). A subset of primary dystonias is autosomal dominant
with a DYT1 gene mutation (GAG deletion in chromosome 9 prominently expressed in the substantia nigra) that leads to childhood onset of primary
dystonia. Abnormal basal ganglia circuitry leads to imbalance of both the indirect and direct pathways with overactivity of the direct pathway, net
decrease in GPi activity, and increase in thalamocortical activation.
Clinical Features
The key characteristics of dystonia include abnormal movements with or without tremor. Abnormal dystonic movements are consistently Page
Chapter 38: Neurosurgery, Timothy R. Smith directional
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and patterned and involve the same muscle groups, leading to twisting of extremities, trunk, or both, and are usually made worse by voluntary
movement. Dystonias are classified based on affected location (generalized, focal, segmental, multifocal, hemidystonia), age of onset (early or late), or
etiology (primary/idiopathic or secondary/symptomatic). Secondary dystonias include dystonia-plus syndromes, nondegenerative disorders
with a DYT1 gene mutation (GAG deletion in chromosome 9 prominently expressed in the substantia nigra) that leads to childhood onset of primary
dystonia. Abnormal basal ganglia circuitry leads to imbalance of both the indirect and direct pathways with overactivity of the direct pathway, net
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decrease in GPi activity, and increase in thalamocortical activation.
Clinical Features
The key characteristics of dystonia include abnormal movements with or without tremor. Abnormal dystonic movements are consistently directional
and patterned and involve the same muscle groups, leading to twisting of extremities, trunk, or both, and are usually made worse by voluntary
movement. Dystonias are classified based on affected location (generalized, focal, segmental, multifocal, hemidystonia), age of onset (early or late), or
etiology (primary/idiopathic or secondary/symptomatic). Secondary dystonias include dystonia-plus syndromes, nondegenerative disorders
associated with other movement disorders (eg, DOPA-responsive dystonia), heterodegenerative dystonias (eg, PD, Wilson disease), and those caused
by CNS insults (eg, stroke, drugs).
Treatment
Medical treatment seeks to relieve symptoms rather than modify the disease course. Anticholinergics, levodopa, baclofen, and neuroleptics are used
for generalized treatment. Botulinum toxin has also shown great results, with focal treatment response rates of 70%-100%. Surgical treatment is
reserved for medically refractory patients and requires that patients undergo a trial of levodopa to rule out patients with dopamine-responsive
dystonia. The best candidates are those with primary dystonia with a DYT1 mutation, segmental cervical dystonia, or hemidystonia not responsive to
medical treatment, whereas patients with secondary dystonia have much decreased success rates. The preferred surgical treatment is GPi DBS, with
improvements of 45%-75% in primary dystonia patients (vs 10%-30% in secondary dystonia). Lesionectomies have been previously performed with
both thalamotomy and pallidotomy but are not currently a preferred treatment.
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Yu H, Neimat JS: The treatment of movement disorders by deep brain stimulation. Neurotherapeutics . 2008;5(1):26–36. [PubMed: 18164481]
INTERVERTEBRAL DISK DISEASE

The intervertebral disks are fibrocartilaginous joints that connect adjoining vertebral bodies. They consist of an outer annulus fibrosis and an inner
nucleus pulposis and serve not only to hold the two vertebral bodies together, but also to help cushion the weight and shock movement of the upper
vertebral body on the lower one.
Intervertebral disks degenerate and dehydrate with age. Coupled with degeneration of the facets, this general process is termed spondylosis. The
process is common and to a certain extent should be expected with age. About 20% of teenagers have signs of mild disk degeneration, whereas by age
70, approximately 60% of disks are severely degenerated. The degenerated and dehydrated disks do not distribute load in the same way and have
alterations in their biomechanics. They do not maintain their height under load-bearing conditions, and as a consequence, more stress is placed on
the vertebral bodies and adjacent facet joints. This promotes pain, inflammation, ligamentous hypertrophy, and reactionary osteophyte formation.
The resulting ligamentous hypertrophy can eventually lead to crowding of the central spinal canal or the exiting neural foramina and neurologic
compression. Neural compression can also occur when the degenerated annulus of the disk fissures, allowing the inner nucleus pulposis to herniate
out and compress nearby nerves. Disk degeneration can also lead to spondylolisthesis, or subluxation of one vertebral body over another.Page 78 / 93
Degenerative disk disease occurs at all levels of the spine, but the lumbar spine and cervical spine are more commonly impacted as they are more
mobile.
process is common and to a certain extent should be expected with age. About 20% of teenagers have signs of mild disk degeneration, whereas by age
70, approximately 60% of disks are severely degenerated. The degenerated and dehydrated disks do not distribute loadInternational Medical University
in the same way and have
alterations in their biomechanics. They do not maintain their height under load-bearing conditions, and as a consequence, more stress is placed on
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the vertebral bodies and adjacent facet joints. This promotes pain, inflammation, ligamentous hypertrophy, and reactionary osteophyte formation.
The resulting ligamentous hypertrophy can eventually lead to crowding of the central spinal canal or the exiting neural foramina and neurologic
compression. Neural compression can also occur when the degenerated annulus of the disk fissures, allowing the inner nucleus pulposis to herniate
out and compress nearby nerves. Disk degeneration can also lead to spondylolisthesis, or subluxation of one vertebral body over another.
Degenerative disk disease occurs at all levels of the spine, but the lumbar spine and cervical spine are more commonly impacted as they are more
mobile.
CERVICAL SPINE
Degenerative disk disease in the cervical spine is a common finding and frequently features a component of at least some axial neck pain. If there is
associated neural compression, symptoms depend on the affected neural structure, with compression of the nerve root leading to radiculopathy and
compression of the spinal cord leading to myelopathy.
Symptoms & Signs
Degenerative disease of the cervical spine often presents with a history of neck pain, which may either be abrupt, in the case of disk rupture, or slowly
progressive. There is often a loss of cervical lordosis (the normal, backward, C-shaped curvature of the neck), which may be related to muscle spasm or
to deformity from chronic degeneration. The characteristic finding in radiculopathy is a sharp, burning pain that radiates down the arm, following a
dermatomal distribution. This pain may be exacerbated when the patient tilts the head toward the side of the pain, crowding the neural foramina on
the affected side (Spurling maneuver). Indeed, the patient may habitually tilt the head to the opposite side to reduce the pain. Numbness can also often
be seen in the same dermatomal pattern, and weakness in the correlating myotome can also sometimes be witnessed. Sensory disturbances
(paresthesias, numbness, or allodynia) tend to occur in the terminal distribution of the involved dermatome (ie, in the fingers rather than the proximal
arm). Weakness from the radiculopathy can occur in the correlating myotome to the pain. Weakness from radiculopathy is often partial or incomplete,
since nearly all muscles are innervated by more than one spinal nerve. Profound weakness, atrophy, and muscle fasciculations are rare in
radiculopathy, except in very long-standing cases. The presence of these findings should generate suspicion of a peripheral nerve lesion.
C5 radiculopathy (typically resulting from pathology at the C4-5 level) involves pain radiating into the shoulder (following the distribution of the C5
dermatome). Patients may exhibit weakness of shoulder abduction (deltoids). C6 radiculopathy (as from a C5-6 herniated disk) typically involves pain
radiating from the neck into the lateral aspect of the arm and possibly the first two digits. Patients may present with biceps weakness. C7 nerve root
compression (from C6-7 pathology) often involves pain radiating from the neck into the back of the shoulder, through the arm, and into the middle
finger. Weakness can sometimes be detected in the triceps or in wrist extension. C8 radiculopathy (from C7-T1 pathology) can present with pain and
numbness radiating into the medial forearm and fourth and fifth digits. Weakness can occasionally be seen in the hand grip. The lower cervical spine
(particularly C5-6 and C6-7) are more commonly affected than the upper cervical spine.
In advanced cases of degenerative disk disease of the cervical spine, signs of myelopathy may develop. These include hyperreflexia, loss of dexterity
(eg, difficulty with cooking or buttoning up one’s shirt), spasticity, gait disturbance, and sensory disturbances of the upper and lower extremities.
Diagnostic Studies
Not all patients with neck pain require imaging. However, those with prolonged and refractory symptoms, those with increased risk for more serious
conditions (eg, tumor or infection), and those with any neurologic deficit should warrant further investigation.
MRI is the primary modality to evaluate potential operative cases (Figure 38–25). Unless infection or tumor is suspected, typically the study need not be
performed with gadolinium contrast. MRI allows for the best differentiation between the nerves, CSF, and any potentially compressive structures. Care
should be taken in interpretation, as radiographic abnormalities are very common and not always symptomatic. Instead, key to establishing a
diagnosis are radiographic findings that correlate with clinical findings (eg, evidence of C5 nerve root compression on MRI in combination with C5
radiculopathy on clinical exam). In cases where an MRI is not able to be obtained, CT myelography can be performed. In CT myelography, a lumbar
puncture is performed and contrast dye is injected into the thecal space, allowing for a delineation of the CNS and any compression thereof.
Figure 38–25.
Sagittal T2-weighted magnetic resonance imaging of cervical spine showing multilevel disk protrusions causing central canal stenosis and cord signal
change.
Figure 38–25.
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Sagittal T2-weighted magnetic resonance imaging of cervical spine showing multilevel disk protrusions causing central canal stenosis and cord signal
change.
Additional studies that may be of use include plain x-ray films, which can be used to assess for bony alignment of the cervical spine. Similar to their use
in the trauma setting, flexion-extension films can also determine any instability as well. CT scans also have a use in perioperative planning, as they
provide superior evaluation of the bony anatomy.
Electrodiagnostic studies, particularly electromyography (EMG), may be useful in diagnosing radiculopathy. Nerve conduction studies alone are of little
value in identifying radiculopathy and are generally normal, even with severe compression of a nerve root. EMG, on the other hand, is more sensitive
and can help confirm dysfunction isolated to a particular nerve root. Unfortunately, EMG will not reliably detect fibrillations in muscles until at least 3-4
weeks following the onset of radiculopathy and thus may lead to false-negative studies if the test is performed too soon. Even when performed after an
appropriate waiting period, EMG findings may be normal in upward of 50% of cases of spinal nerve compression in patients with only complaints of
pain without weakness.
In general, severe weakness and muscle atrophy are suggestive of a peripheral nerve lesion, whereas early loss of a reflex (biceps, triceps) suggests
radiculopathy. Other conditions that may mimic cervical degenerative disk disease include myocardial infarction, idiopathic brachial plexitis
(Parsonage-Turner syndrome), or inflammatory conditions such as ankylosing spondylitis or sarcoidosis. Local conditions affecting the shoulder (eg,
rotator cuff tears, acromial bursitis) must also be ruled out. Patients with any atypical features to their pain, such as fever, night sweats, unexplained
weight loss, a history of chronic infections or immunosuppression, or a history of metastatic cancer, should also be evaluated for possible infection or
tumor.
Most conditions that cause pain in the cervical spine (eg, exacerbations of degenerative arthritis, muscle spasm, or minor trauma) are self-limiting and
ultimately do not require operation. Acute neck pain may be treated with gentle exercise or a mobilization program, moist heat, or a soft collar to help
muscle relaxation. Anti-inflammatory medications are also useful in this regard. For persistent neck pain, intermittent traction is sometimes helpful,
either through physical therapy or with a home traction kit. The vast majority of patients improve with medical management alone, although many
continue to have mild symptoms they ultimately learn to manage. Page 80 / 93
Neck pain itself generally responds poorly to operative management, and surgery is typically more targeted at the symptoms of neural compression.
Surgical management of cervical degenerative disk disease should be reserved for cases failing medical management and where there is neurologic
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Most conditions that cause pain in the cervical spine (eg, exacerbations of degenerative arthritis, muscle spasm, or minor trauma) are self-limiting and
ultimately do not require operation. Acute neck pain may be treated with gentle exercise or a mobilization program, moist heat, or a soft collar to help
muscle relaxation. Anti-inflammatory medications are also useful in this regard. For persistent neck pain, intermittent traction is sometimes helpful,
either through physical therapy or with a home traction kit. The vast majority of patients improve with medical management alone, although many
continue to have mild symptoms they ultimately learn to manage.
Neck pain itself generally responds poorly to operative management, and surgery is typically more targeted at the symptoms of neural compression.
Surgical management of cervical degenerative disk disease should be reserved for cases failing medical management and where there is neurologic
compression (leading to either myelopathy or radiculopathy). Operative management of the cervical spine involves decompression of the spinal cord
or nerve roots, with or without fusion. The cervical spine may be approached either anteriorly or posteriorly, with many pathologies approachable
through either route and thus dependent on surgeon preference. Complex cases may require both approaches (combined anterior/posterior).
Options for anterior correction include removal of the disk with either fusion of the adjoining vertebral bodies (anterior cervical discectomy and
fusion) or placement of an artificial disk (anterior cervical arthroplasty). It is also possible to drill away the vertebral body itself, with placement of a
replacement strut for support (anterior cervical corpectomy). Posterior approaches include removal of the lamina (cervical laminectomy) with or
without fusion, removal of lamina with replacement at the end of the surgery (cervical laminoplasty), and opening of the neural foramina (posterior
cervical foraminotomy).
Outcomes from surgery are highly dependent on patient selection and the specific scenario. Well-selected patients, with symptoms well-correlated
with imaging findings, have a favorable prognosis, with > 80% of patients reporting long-term satisfaction.
THORACIC DISK DISEASE
Thoracic disk herniations are rare, with an incidence between 0.25% and 0.75% of all disk herniations. Often there is a delay in diagnosis because of
poorly defined symptoms and the lack of objective findings on physical exam.
Patients can present with symptoms of axial pain, radiculopathy, myelopathy, or some combination of the three. The axial pain may be described as
dull, aching, burning, stabbing, or cramping. Load bearing, activity, or Valsalva will often exacerbate the pain. Radicular symptoms generally present in
the appropriate dermatomal band. Myelopathy can present as paraparesis, but more often presents with a vague history of lower extremity weakness,
gait instability, heaviness, stiffness, or numbness. Bowel and bladder complaints can occur as well.
Treatment for symptomatic lesions is surgical and is directed at alleviating pain or preventing progression of a neurologic deficit. Surgery is more
complex than equivalent cervical or lumbar cases as anterior approaches are limited by the thoracic or abdominal viscera and posterior approaches
are limited by the spinal cord. Nevertheless, options exist from both anterior and posterior approaches (including transthoracic, lateral extracavitary,
costotransversectomy, and transpedicular). In cases of a thoracic disk herniation, a strictly dorsal midline approach (laminectomy) offers poor
exposure of the disk and has a high risk of neurologic injury.
LUMBAR SPINE
Symptoms & Signs
As in the cervical spine, degenerative disk disease of the lumbar spine can present as a combination of back pain, radiculopathy, and myelopathy.
Similarly, radiculopathy can be identified because symptoms will localize to a particular dermatome and possibly the associated myotome as well.
There are a few differences that bear consideration, however.
First, myelopathy is less common in the lumbar spine because the spinal cord itself terminates at the L1-2 level. Instead, any stenosis of the central
spinal canal further down may present as neurogenic claudication. Just as in vascular claudication, these patients complain of leg pain upon walking
that is alleviated upon sitting down. However, in neurogenic claudication, this is due to nerve compression from the spinal canal narrowing when the
back is extended. In addition, severe spinal canal compression below the level of the spinal cord can produce cauda equina syndrome. This is
characterized by the presence of saddle anesthesia, bowel and bladder dysfunction, lower extremity weakness (typically severe), and severe pain.
Because many patients, especially those with an impaired mental status, can complain of some combination of these symptoms, the diagnosis can
sometimes be difficult. Signs that should elevate the suspicion for cauda equina syndrome include a poor rectal tone, an absent bulbocavernosus
reflex, and an absent anal wink.
In the lumbar spine, a straight leg raise can be useful in helping diagnose herniated lumbar disks. In such a scenario, the patient presents with
radiating leg pain suspicious for a herniated disk. The straight leg raise is positive if raising the affected leg to an angle of 30 degrees reproduces the
radiating leg pain. The contralateral leg is then tested in a similar fashion to see if raising it will reproduce the pain in the original leg. These two are
then correlated, as a positive ipsilateral straight leg raise is sensitive but not specific, while a positive contralateral straight leg raise is specific but not
sensitive.
As in the cervical spine, determination of dermatomal and/or myotomal pattern of symptoms is key for evaluating radiating pain. Compression of the
Because many patients, especially those with an impaired mental status, can complain of some combination of these symptoms, the diagnosis can
sometimes be difficult. Signs that should elevate the suspicion for cauda equina syndrome include a poor rectal tone, an absent bulbocavernosus
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reflex, and an absent anal wink.
In the lumbar spine, a straight leg raise can be useful in helping diagnose herniated lumbar disks. In such a scenario, the patient presents with
radiating leg pain suspicious for a herniated disk. The straight leg raise is positive if raising the affected leg to an angle of 30 degrees reproduces the
radiating leg pain. The contralateral leg is then tested in a similar fashion to see if raising it will reproduce the pain in the original leg. These two are
then correlated, as a positive ipsilateral straight leg raise is sensitive but not specific, while a positive contralateral straight leg raise is specific but not
sensitive.
As in the cervical spine, determination of dermatomal and/or myotomal pattern of symptoms is key for evaluating radiating pain. Compression of the
L4 nerve root (L3/4 herniation) may cause weakness of knee extension (quadriceps) and numbness in the anterior thigh and medial ankle.
Compression of the L5 nerve root (L4/5 herniation) may precipitate weakness of the extensor hallucis longus and ankle dorsiflexion (tibialis anterior)
and numbness of the dorsal foot and first web space. Finally, compression of the S1 nerve root (L5/S1 herniation) may lead to weakness of ankle
plantarflexion (gastrocnemius) and numbness in the lateral ankle and plantar side of the foot.
Imaging Studies
As in the cervical spine, MRI has become the gold standard for diagnosis of degenerative disk disease in the lumbar spine (Figure 38–26). It provides
superior evaluation of the soft tissue structures and is used to see if there is a unified clinical and radiographic explanation for the patient’s symptoms.
CT myelography can be used in its stead when MRI is contraindicated.
Figure 38–26.
Sagittal T2-weighted magnetic resonance imaging of lumbar spine showing multilevel disk bulges, ligamentum flavum hypertrophy, and retrolisthesis
at L2-3.
CT scans of the lumbar spine are also of significant utility as they identify the nature of previous spine surgery (which patients frequently cannot speak
exactly to), the quality of the bones, and the exact bony anatomy. Standing plain-film x-rays are also useful, as increasingly there is an awareness that
the balance of the spine in the sagittal plane can have a significant contribution to back pain. These films can help determine any deformities in the
spine’s overall alignment and may suggest alterations in surgical strategy for certain patients.
Figure 38–26.
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Sagittal T2-weighted magnetic resonance imaging of lumbar spine showing multilevel disk bulges, ligamentum flavum hypertrophy, and retrolisthesis
at L2-3.
CT scans of the lumbar spine are also of significant utility as they identify the nature of previous spine surgery (which patients frequently cannot speak
exactly to), the quality of the bones, and the exact bony anatomy. Standing plain-film x-rays are also useful, as increasingly there is an awareness that
the balance of the spine in the sagittal plane can have a significant contribution to back pain. These films can help determine any deformities in the
spine’s overall alignment and may suggest alterations in surgical strategy for certain patients.
As mentioned previously, EMG can be useful when the diagnosis of radiculopathy remains unclear. EMG can help evaluate if pain is radiculopathic in
nature and help isolate which myotome is the affected one.
Medical Treatment
The natural history of most acute-onset back pain and radiculopathy associated with disk herniations is that of improvement over time, with significant
improvement in the first 4-6 weeks after onset of symptoms. Therefore, conservative measures are recommended initially for patients who present
without neurologic deficit. Conservative measures are directed toward initially limiting physical activity, including a brief period of bed rest followed by
a gradual exercise program. It is also important for patients to modify their types of movement, for example, to limit heavy lifting, twisting, or bending.
Physical therapy can be useful after the acute period for instruction in abdominal and back musculature strengthening exercises. Although of limited
utility in radiculopathy, more generalized back pain related to degenerative spine disease can also respond to external bracing.
The core of medical treatment is nonsteroidal anti-inflammatory drugs (NSAIDs). Oral steroids (eg, solumedrol dose pack) also may be useful in the
acute setting, and epidural steroid injections and narcotics may be helpful in alleviating pain.
Surgical Treatment
Patients who are refractory to conservative measures or those who present with acute neurologic motor deterioration warrant surgical attention.
Surgery is also indicated in patients who fail the conservative measures outlined earlier and continue to suffer from debilitating pain.
For herniated lumbar disks, a microdiscectomy can be performed where the extruded disk material and some of the underlying nucleus pulposus is
removed for decompression. Patients primarily presenting with symptoms of stenosis may receive laminectomies, where the lamina and
The core of medical treatment is nonsteroidal anti-inflammatory drugs (NSAIDs). Oral steroids (eg, solumedrol dose pack) also may be useful in the
acute setting, and epidural steroid injections and narcotics may be helpful in alleviating pain. Access Provided by:
Surgical Treatment
Patients who are refractory to conservative measures or those who present with acute neurologic motor deterioration warrant surgical attention.
Surgery is also indicated in patients who fail the conservative measures outlined earlier and continue to suffer from debilitating pain.
For herniated lumbar disks, a microdiscectomy can be performed where the extruded disk material and some of the underlying nucleus pulposus is
removed for decompression. Patients primarily presenting with symptoms of stenosis may receive laminectomies, where the lamina and
hypertrophied ligamentum flavum in the posterior aspect of the spinal canal are removed.
The use of instrumented spinal fusion has expanded significantly in recent years and remains an area of controversy. It is indicated in patients who
present with signs and symptoms of spinal instability or when the spine is deliberately destabilized as part of the surgery (eg, in the process of
removing bone to correct a deformity or the complete removal of a facet joint or disk space to facilitate a decompression). Many options exist for
fusion, including anterior approaches (eg, anterior lumbar interbody fusion) and posterior approaches (eg, posterolateral fusion with pedicle screws).
Prognosis
Degenerative disk disease in the lumbar spine has varied presentations and sometimes overlapping pathologies. As such, prognosis varies
significantly with the condition and presenting symptoms. Generally, acute exacerbations of degenerative spine disease have a favorable prognosis,
and most episodes will recover well with conservative management. However, more episodes will likely occur in the future as the patient continues to
age.
Outcomes from surgical treatment depend greatly on patient selection and indications for surgery. As a broad generalization, surgery for neural
decompression carries a significantly better prognosis than surgery for back pain. Thus, patients undergoing surgery for herniated lumbar disks
generally fare well, although approximately 10% may experience reherniations.
Ambrossi GLG, McGirt MJ, Sciubba DM, et al: Recurrent lumbar disc herniation after single-level lumbar discectomy: incidence and healthcare cost
analysis. Neurosurgery. 2009;65(3):574–578. [PubMed: 19687703]
Atlas SJ, Keller RB, Wu YA, et al: Long-term outcomes of Surgical and nonsurgical management of sciatica secondary to a lumbar disc herniation: 10
year results from the Maine lumbar spine study. Spine. 2005;30(8):927–935. [PubMed: 15834338]
Boselie TF, Willems PC, van Mameren H, et al: Arthroplasty versus fusion in single-level cervical degenerative disc disease. Cochrane Database Syst
Rev. 2012;9:CD009173.
Jacobs W, Van der Gaag NA, Tuschel A, et al: Total disc replacement for chronic back pain in the presence of disc degeneration. Cochrane Database
Syst Rev . 2012;9:CD008326.
Kovacs FM, Urrutia G, Alarcon JD: Surgery versus conservative treatment for symptomatic lumbar spinal stenosis: a systematic review of randomized
controlled trials. Spine. 2011;36:E1335–E1351. [PubMed: 21311394]
Matz PG, Anderson PA, Holly LT, et al: The natural history of cervical spondylotic myelopathy. J Neurosurg Spine . 2009;11:104–111. [PubMed:
19769489]
CSF DIVERSION FOR HYDROCEPHALUS

Kyle Wu, MD, and Timothy R. Smith, MD, PhD
OVERVIEW, EPIDEMIOLOGY, & PATHOPHYSIOLOGY
Hydrocephalus is a common diagnosis in both adult and pediatric patients. Most often, this disease is chronically treated with an implanted catheter
system to divert CSF from the ventricles of the brain to an alternative absorptive space such as the pleural or peritoneal space for absorption, known as
a CSF shunt. Endoscopic third ventriculostomy is a surgical technique that is commonly used as well that obviates the placement of hardware if
successful. The disease is estimated to occur at an incidence of 1 per 1000 live births and has a prevalence of approximately 0.5% within the United
States. Shunt failure is common, occurring in up to 30%-35% of individuals within 1 year of initial shunt placement, with about 1% of shunt
Chapter 38: Neurosurgery, Timothy R. Smith failures
Page 84 / 93
being fatal. Familiarity with the diagnosis and treatment of shunt failure is therefore warranted for providers caring for patients in whom a CSF shunt is
present.
OVERVIEW, EPIDEMIOLOGY, & PATHOPHYSIOLOGY
Hydrocephalus is a common diagnosis in both adult and pediatric patients. Most often, this disease is chronically treated with an implanted catheter
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system to divert CSF from the ventricles of the brain to an alternative absorptive space such as the pleural or peritoneal space for absorption, known as
a CSF shunt. Endoscopic third ventriculostomy is a surgical technique that is commonly used as well that obviates the placement of hardware if
successful. The disease is estimated to occur at an incidence of 1 per 1000 live births and has a prevalence of approximately 0.5% within the United
States. Shunt failure is common, occurring in up to 30%-35% of individuals within 1 year of initial shunt placement, with about 1% of shunt failures
being fatal. Familiarity with the diagnosis and treatment of shunt failure is therefore warranted for providers caring for patients in whom a CSF shunt is
present.
In pediatric patients, hydrocephalus is primarily seen in patients with a history of intraventricular hemorrhage, meningitis, congenital cranial
malformation, brain tumor, or myelomeningocele. In adults, diseases such as SAH (often from ruptured cerebral aneurysm), brain tumor, or head
trauma may lead to development of hydrocephalus. Alternatively, idiopathic intracranial hypertension (IIH; or pseudotumor cerebri) may affect
patients, leading to blindness. In older adults, “normal-pressure” hydrocephalus is a potentially treatable cause of dementia.
Hydrocephalus results from an interruption in CSF circulation and drainage. The choroid plexus of the brain’s ventricular system makes up about 80%
of total adult CSF production, resulting in the generation of about 20 mL/h by an active ion pump–dependent process. The remainder is thought to be
generated by general metabolic processes occurring within the brain, as well as via ependymal cells lining the ventricles. Importantly, the production of
CSF is minimally affected by the ICP over a wide range of physiologic values. Thus, increases in ICP resulting from increasing CSF volume do not act to
significantly slow or stop further production of CSF until relatively high ICP is reached. Once produced, spinal fluid is hypothesized to move in an
oscillatory fashion out of the ventricular system and into the subarachnoid space around the brain and spinal cord. From there, it is reabsorbed mainly
by passive, pressure-dependent mechanisms into the cerebral venous sinuses via arachnoid granulations, and some is even reabsorbed into the brain
parenchyma itself.
The vast majority of hydrocephalus results from interruption in this process of CSF egress. This can occur by either of two mechanisms, often classified
as either noncommunicating/obstructive, if the blockage to CSF outflow prevents an outlet for CSF drainage from the ventricular system, or
communicating, if reabsorption is reduced or prevented at the level of the arachnoid granulations. Obstructive etiologies such as tumors,
aqueductal stenosis, intraventricular cysts, or even strictures are mainly diagnosed with radiologic imaging. Communicating causes can occur
following SAH (after cerebral aneurysm rupture), infection, or tumor spread within the subarachnoid space. This distinction between the mechanism
of hydrocephalus is important in determining the expected course of the pathology and appropriate treatment. It is also vital for determining the safety
of performing a lumbar puncture. If a patient has obstructive hydrocephalus, a lumbar puncture may be unsafe because of the potential for creating
differential pressures between the cranial and spinal spaces after drainage of CSF from the spinal canal. Radiographically, hydrocephalus is
sometimes assumed to be communicating when all four ventricles are dilated, as opposed to only the ventricles proximal to the obstruction. However,
blockage of CSF outflow from the fourth ventricle to the subarachnoid space at the level of the foramens Luschka or Magendie could produce the same
dilated quad-ventricular radiologic picture despite an obstructive physiology. Consultation with a neurosurgeon or neurologist may be helpful if the
situation is unclear.
CLINICAL PRESENTATION & ASSESSMENT
The relative difference between the volume of CSF produced and reabsorbed and the relative compressibility of the brain determine the severity and
type of clinical symptoms and signs at presentation. Slower buildup of CSF or accumulation within a brain more compliant to compression (such as
might occur at the extremes of age or with alcoholism) produces a more protracted course of symptoms. This is in contrast to rapid CSF accumulation
within a poorly compressible brain (eg, a young adult’s brain) leading to acute neurologic decline. Whether presenting initially or after treatment
failure, the symptoms of hydrocephalus can be grouped into two main categories: those related to acute increased ICP, usually seen with rapid
accumulation of CSF in a poorly compliant brain, and those related to chronic deformation of the nervous system by slowly accumulating CSF.
Acute, rapidly progressive hydrocephalus presents with headaches, nausea, and vomiting. As it progresses further, patients may experience decreased
levels of consciousness and eventually brainstem herniation and coma. Some patients may present with the loss of ability to look upward as part of a
constellation of symptoms, known as Parinaud syndrome. This syndrome occurs due to compression of the midbrain tectum resulting in upgaze
paralysis, pseudo-Argyll Robertson pupils, convergence-retraction nystagmus, and conjugate downgaze. In some cases, patients may also lose the
ability to abduct their eyes due to cranial nerve VI palsy from increased ICP (due to traction on the nerve as it enters the Dorello canal within the skull
base). If an open fontanelle is present in the infant, it will be tense and raised. Scalp veins may be distended as well due to decreased blood drainage
via the intracranial venous sinuses and subsequent shunting of blood through the valveless scalp veins.
Chronic hydrocephalus presents in a slowly progressive nature that is typically more insidious in nature. In children, an inappropriately large head size
that is increasing rapidly over time may suggest hydrocephalus. This may be accompanied by fussiness, frequent vomiting, failure to thrive, and
eventually lethargy or coma. In adults, chronic diseases such as IIH may present with a slow but steady visual decline, chronic headaches, nausea,
vomiting, dizziness, or tinnitus. This disease often afflicts obese individuals and is associated with certain rheumatologic, endocrinologic, or
inflammatory diseases, as well as use of certain medications (eg, birth control, retinoids, lithium, or doxycycline). In the elderly, normal-pressure
hydrocephalus presents with the triad of dementia, gait disturbance, and urinary incontinence. The order of symptom onset is important in diagnosis
of this disease, with gait issues presenting first, followed by urinary incontinence and finally subacute-onset dementia.
base). If an open fontanelle is present in the infant, it will be tense and raised. Scalp veins may be distended as well due to decreased blood drainage
via the intracranial venous sinuses and subsequent shunting of blood through the valveless scalp veins.
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Chronic hydrocephalus presents in a slowly progressive nature that is typically more insidious in nature. In children, an inappropriately large head size
that is increasing rapidly over time may suggest hydrocephalus. This may be accompanied by fussiness, frequent vomiting, failure to thrive, and
eventually lethargy or coma. In adults, chronic diseases such as IIH may present with a slow but steady visual decline, chronic headaches, nausea,
vomiting, dizziness, or tinnitus. This disease often afflicts obese individuals and is associated with certain rheumatologic, endocrinologic, or
inflammatory diseases, as well as use of certain medications (eg, birth control, retinoids, lithium, or doxycycline). In the elderly, normal-pressure
hydrocephalus presents with the triad of dementia, gait disturbance, and urinary incontinence. The order of symptom onset is important in diagnosis
of this disease, with gait issues presenting first, followed by urinary incontinence and finally subacute-onset dementia.
If the patient has already had a CSF shunt placed for such conditions, then additional signs and symptoms to consider include those of hardware
infection, such as a stiff neck, fever, confusion, photophobia, nausea, vomiting, abdominal pain, or redness around the device. If the device has broken
or malfunctioned, then CSF may accumulate around the disconnected portion of the shunt or shunt valve. If the distal cavity into which a shunt is
placed fails to absorb spinal fluid, there may be symptoms related to this, such as abdominal pain from a large sterile intraperitoneal fluid collection
(eg, pseudocyst) or intra-abdominal abscess.
The headaches associated with hydrocephalus and/or CSF shunt failure often have morning predominance (occurring after a period of prolonged
recumbency) or worsen with Valsalva maneuver (due to transiently increased ICP).
Patients presenting with the clinical features described earlier, especially with a history of a CSF shunt in place for prior treatment of hydrocephalus
must be promptly evaluated with imaging studies, usually a CT or MRI scan of the brain. In the setting of a possible shunt failure, it is critical to have
prior images available for comparison. Patients with working shunts often have ventricles that are smaller than normal. Radiology reports reading
“normal ventricular size” or “no evidence of shunt failure” have been demonstrated to correlate poorly with final diagnosis. Additional studies that can
be useful include plain radiographs of the shunt to look for kinks, break, or disconnections. Imaging of the cavity into which the CSF shunt is draining,
such as by ultrasound or CT of the abdomen, may be useful in situations where pseudocyst or abscess is suspected. If the diagnosis remains in
question, more invasive diagnostic studies may be necessary, usually as directed by a neurosurgical consultant. These can include a CSF shunt tap, in
which a portion of the shunt that sits under the scalp is accessed percutaneously similar to a vascular access port. CSF can be removed and sent for
analysis and ICP measured during this bedside procedure. In addition, lumbar puncture offers similar benefits; however, there is less of a risk of
infecting or damaging the shunt when using this method. It is helpful to know that a high percentage of shunt failures occur within the first 2 years after
shunt placement, peaking within the first 6 months and steadily declining thereafter. In children, young age at presentation also appears to be a risk
factor for repeated shunt failure. Similarly, most shunt infections occur within 1 year of shunt placement. However, despite these epidemiologic data, a
perfectly predictive clinical decision algorithm remains elusive, and a low threshold for obtaining imaging studies and appropriate expert
consultations is warranted.
MANAGEMENT
Progressive hydrocephalus must be dealt with in a timely manner to minimize neurologic deterioration. As noted earlier, the rate of pathologic CSF
accumulation may vary and produce variable clinical symptoms. However, because of the potential for acute and fatal deterioration from
hydrocephalus, the initial presumption should be that rapid intervention is required, subject to reconsideration after all necessary data are available.
Medical management of hydrocephalus may be appropriate in the management of IIH and, in some patients, after SAH. It may also be used in the
initial management of hydrocephalus in neonates following intraventricular hemorrhage. The diuretics acetazolamide and furosemide are both used
in this context. They act to decrease CSF production by inhibiting carbonic anhydrase, which decreases CSF production at the choroid plexus.
Furosemide is also thought to decrease cerebral sodium uptake, thus producing an additive effect when the two medications are used in combination.
However, randomized controlled trials of medical therapy in newborns demonstrated no reduction in the need for subsequent shunt placement for
patients after medical therapy. If medical management is chosen, patients must be closely monitored for electrolyte imbalances and acetazolamide
toxicity (acute gastritis, paresthesias, nephrocalcinosis, and drowsiness). It should be noted that with the exception of IIH, protracted use of diuretics
to manage hydrocephalus is rarely successful, and in most cases, surgical therapy is indicated without a trial of diuretics.
Intraventricular administration of fibrinolytic agents (eg, streptokinase) has also been investigated as a treatment for newborns with posthemorrhagic
hydrocephalus. However, a systematic review of randomized trials failed to show any benefit in terms of reducing shunt requirement or death.
Furthermore, secondary intraventricular hemorrhage is a potential complication of this treatment.
Temporary drainage techniques include serial lumbar punctures or ventricular taps (either trans-fontanelle or via a surgically created subgaleal
fluid collection connected to the ventricle via a shunt). Other techniques include continuous drainage by an external ventriculostomy catheter or via
serial taps of an implanted reservoir in communication with the ventricles. These techniques are used when etiology and natural history suggest
possible resolution of the hydrocephalus over time, as may occur in intraventricular hemorrhage of the newborn or SAH. In addition, temporary
diversion is appropriate when the patient’s medical condition precludes definitive treatment. Page 86 / 93
Endoscopic third ventriculostomy (ETV) is an alternative to CSF shunt placement for the treatment of some forms of hydrocephalus. During the
endoscopic procedure, a perforation is made in the floor of the third ventricle (most commonly through the tuber cinereum) communicating the
hydrocephalus. However, a systematic review of randomized trials failed to show any benefit in terms of reducing shunt requirement or death.
Furthermore, secondary intraventricular hemorrhage is a potential complication of this treatment. International Medical University
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Temporary drainage techniques include serial lumbar punctures or ventricular taps (either trans-fontanelle or via a surgically created subgaleal
fluid collection connected to the ventricle via a shunt). Other techniques include continuous drainage by an external ventriculostomy catheter or via
serial taps of an implanted reservoir in communication with the ventricles. These techniques are used when etiology and natural history suggest
possible resolution of the hydrocephalus over time, as may occur in intraventricular hemorrhage of the newborn or SAH. In addition, temporary
diversion is appropriate when the patient’s medical condition precludes definitive treatment.
Endoscopic third ventriculostomy (ETV) is an alternative to CSF shunt placement for the treatment of some forms of hydrocephalus. During the
endoscopic procedure, a perforation is made in the floor of the third ventricle (most commonly through the tuber cinereum) communicating the
ventricles with the subarachnoid space that exists in the basal cisterns anterior to the brainstem. ETV is currently used as the initial treatment of choice
in cases of obstructive hydrocephalus that are not treatable by removal of the obstruction itself and is curative in 80% of properly selected patients,
avoiding the need for shunt placement. It is also used frequently in pediatric patients with aqueductal stenosis (narrowing of the CSF drainage
pathway) and is combined with choroid plexus cauterization in infants less than 1 year old to achieve superior outcomes. The use of ETV is routinely
performed for nonobstructive causes of hydrocephalus as well, such as post-intraventricular hemorrhage, Dandy-Walker malformation, spina bifida,
encephalocele, or infection, with varying degrees of success. Given its relatively noninvasive nature and the retained ability to place a shunt in cases of
failure, it is sometimes attempted prior to shunt placement as a means of obtaining shunt independence. Similar to shunt epidemiology, ETVs typically
will fail within a 1-year time frame (due to closure of the fenestration); however, the incidence of infection is much lower, as would be expected given
that no implantable hardware is involved.
CSF shunt treatment is indicated in most cases of progressive hydrocephalus, when conservative management and ETV are not indicated or have
previously failed. This procedure involves placement of a mechanical shunt as a means to divert excess CSF from the ventricles or lumbar spinal
subarachnoid space (lumbar peritoneal shunts are used infrequently, mainly in IIH) into other body cavities. CSF shunts consist of three major
components in continuity: a proximal ventricular catheter, a valve that regulates unidirectional CSF flow, and distal tubing to carry the CSF to its
absorption site. Some shunts also have reservoirs at the end of the proximal ventricular catheter that can be percutaneously accessed for diagnostic
purposes, to inject intrathecal medications (ie, chemotherapeutic agents), or as a temporizing measure in case the distal tubing or valve becomes
blocked. Most often, the proximal catheter of the shunt system is placed into one of the lateral ventricles, either in the frontal horn via a frontal
approach or via the atrium of the lateral ventricle via an occipital (posterior) approach. The technique is mainly provider dependent. Shunt systems
may also be used to treat a variety of other conditions such as intracranial cysts or chronic SDHs/hygromas. Shunt valves regulate the amount of fluid
drainage from the proximal catheter. Most are differential pressure systems that respond to increasing fluid pressure by allowing more CSF through
the valve. Various shunts are designed to open in different pressure ranges (eg, low, medium, or high), and some valves can be externally adjusted to
different pressure ranges using a magnet. These adjustable (programmable) valves may be inadvertently reprogrammed by nonmedical external
magnets, and although all systems approved for sale in the United States as of this writing are MRI compatible with 1.5-T magnets, the patient must
have the valve setting checked (most accurately using a skull x-ray orthogonal to the valve) and reset if necessary following an MRI scan. In certain
instances, practitioners may choose to incorporate devices that prevent excessive drainage from the siphoning effect due to a long run of distal tubing
running inferiorly when patients assume an upright posture, a phenomenon that may lead to overdrainage of CSF. The distal portion of the shunt
system follows the valve and is comprised of a long catheter that terminates within a body cavity, allowing for fluid to be adequately absorbed. A
ventriculoperitoneal (VP) shunt, the most commonly used shunt, employs a catheter that terminates in the abdominal cavity so that CSF may be
absorbed by the peritoneum. Alternative sites, in order of decreasing usage, include the pleural space (ventriculopleural shunt) or the cardiac atria
(ventriculoatrial shunt). These alternative sites are chosen most commonly when there is extensive abdominal scarring or adhesions, recent or active
abdominal infection, peritonitis, or morbid obesity that precludes safe peritoneal catheter placement. Major considerations for ventriculopleural
shunts include the risk of developing clinically significant pleural effusions. Patients who receive ventriculoatrial shunts are at increased risk of
septicemia. Other much less common options for distal placement of a ventricular shunt include the gallbladder or ureter/bladder.
Complications from shunt placement during surgery are infrequent and arise in the context of the surrounding anatomy. Ventricular catheters may be
misplaced, resulting in inadequate shunt function or intracranial hemorrhage. Patients undergoing VP shunt may experience bowel perforation or
misplacement into the extraperitoneal space, leading to a CSF collection in the nonabsorptive space and subsequent fistulation through the skin. VP
shunt patients are also at increased risk of developing hernias and bowel obstruction. Patients with ventriculopleural shunts may develop pleural
effusions, leading to shortness of breath and hypoxia. During ventriculoatrial shunt placement, patients may develop cardiac tamponade. Afterward,
these patients are at increased risk for septicemia and pulmonary thromboembolism. When evaluating for shunt complications, one must keep in
mind the unique anatomy of the particular shunt within that patient and integrate a thorough history, physical exam, laboratory studies, radiologic
findings, and possibly invasive diagnostic procedures to make the correct diagnosis.
CSF SHUNT FAILURE
Although the surgical procedure of CSF shunt placement is generally an uncomplicated process, the long-term management of shunted
hydrocephalus is more problematic. Multiple clinical studies in both adult and pediatric patients attest to the high rate of device failure, which includes
entities such as catheter occlusion, valve malfunction, distal catheter kink or fracture, disconnection of components, infection, or excessive drainage.
In young children undergoing first-time shunt placement, roughly one third of shunts will require reoperation in the first year following surgery. Shunt
infection rates in children are as high as 10%-12% and are generally higher in patients with shunts placed at a younger age. Adult patients fair
mind the unique anatomy of the particular shunt within that patient and integrate a thorough history, physical exam, laboratory studies, radiologic
findings, and possibly invasive diagnostic procedures to make the correct diagnosis.
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CSF SHUNT FAILURE
Although the surgical procedure of CSF shunt placement is generally an uncomplicated process, the long-term management of shunted
hydrocephalus is more problematic. Multiple clinical studies in both adult and pediatric patients attest to the high rate of device failure, which includes
entities such as catheter occlusion, valve malfunction, distal catheter kink or fracture, disconnection of components, infection, or excessive drainage.
In young children undergoing first-time shunt placement, roughly one third of shunts will require reoperation in the first year following surgery. Shunt
infection rates in children are as high as 10%-12% and are generally higher in patients with shunts placed at a younger age. Adult patients fair
somewhat better, but approximately 20% will still require reoperation within the first year after shunt failure. Failure rates for devices drops
considerably after the first year or two from surgery, but the threat of failure remains present to some degree as long as the device remains in place.
In evaluating a patient for CSF shunt failure, the above epidemiologic data provide a baseline approximation for the probability of the diagnosis. A
history and physical exam, along with images compared to previous findings, as described earlier, help to guide diagnosis. However, several pitfalls
deserve mention. First, as has been noted earlier, not all patients with CSF shunt failure will demonstrate significant expansion of the ventricular
system. Some patients, particularly those with a long history of shunted hydrocephalus, may present with so-called “slit-ventricle” syndrome, with
severely narrowed ventricles on imaging. The clinical slit-ventricle syndrome presents with episodic severe headaches that are thought to occur due to
intermittent occlusion of the proximal ventricular catheter within the collapsed ventricles. The diagnosis is often confirmed by direct ICP
measurements obtained from a separately placed ICP monitor.
A second group that deserves special attention is children and adults with myelomeningocele. About 70% of patients with this spinal dysraphism will
require treatment for hydrocephalus, most with a CSF shunt. Myelomeningocele is associated with a number of abnormalities of the brainstem and
foramen magnum. These abnormalities allow CSF shunt failure to produce a much wider array of clinical symptoms. As intracranial CSF and pressure
build because of CSF shunt failure, downward pressure on the brainstem may produce lower cranial nerve palsies, including swallowing and breathing
irregularities. Mortality rates are higher and rapidity of neurologic decline more precipitous, likely related to the susceptibility of the hindbrain to
herniation. In addition, CSF shunt failure may precipitate or exacerbate a preexisting spinal syrinx in these patients, producing symptoms related to
spinal cord dysfunction.
Shunt contamination may occur in bacteremic patients, especially in the presence of a ventriculoatrial shunt. It may also arise due to intra-
abdominal infections or surgical procedures with nonsterile spillage, as in the case of a VP shunt. Clinically, the presentation of infection can vary
greatly. It can range from high-grade fever with overt meningitis and symptoms of acute shunt failure to something more insidious, such as a slight
headache, pain along the shunt tract, decreased energy, or subtle recrudescence of the patient’s presenting hydrocephalus symptoms. The presence
of a large intra-abdominal fluid collection in a patient with a VP shunt should raise suspicion of shunt infection, although sterile fluid collections are
not uncommon.
Abdominal surgical procedures in patients with VP shunts raise the issue of how best to manage an intraperitoneal distal shunt catheter during
and after the abdominal procedure. While no validated guideline exists, the literature and the author’s experience suggest that the infection risk is low
during clean and clean-contaminated cases. If an enterotomy or open bladder procedure is to be performed, it is reasonable to relocate the shunt
away from this area once the tubing is identified intraperitoneally and protect it with bacitracin-soaked gauze sponges during the procedure. Other
than routine preoperative antibiotics, expectant management for shunt infection and failure can be practiced, and the shunt does not require
externalization. In situations where there is frank contamination of the shunt catheter in the abdomen, externalization of the shunt would seem
prudent. Laparoscopic procedures have been demonstrated to transiently increase ICP due to increased intra-abdominal pressures, but there is no
body of literature to suggest that this produces identifiable complications, presumably due to its transient nature. Constipation and ileus, conversely,
have been suggested to be a source of at least transient shunt dysfunction due to increased intra-abdominal pressure.
Shunt externalization is most often necessary in cases of distal catheter infection. It involves palpation of the shunt proximal to its entry into the
peritoneum, followed by a sterile prep and, if the patient is awake, local anesthesia. If the shunt has been in place for several years, it may be adherent
to the surrounding tissues and the procedure is then best performed in the operating room under anesthesia. For recently placed shunts, this is less of
an issue and the procedure can be performed at the bedside, assuming a cooperative patient. A cut down is made over the shunt tract, avoiding
laceration of the underlying tubing. The catheter is often encased in a thick sheath of scar that must be carefully opened to gain access to the catheter.
If a specimen of fluid is required from a peritoneal fluid collection, the tubing is then cut and aspirated distally. In a large pseudocyst, a liter or more of
fluid may be withdrawn. The distal catheter is then withdrawn from the patient and the catheter itself sent for culture. The remaining proximal tubing
should be observed to confirm CSF drainage and connected to a sterile, enclosed CSF drainage system. Patients with externalized shunts drain
isotonic CSF and may suffer hyponatremia if appropriate fluid and electrolyte repletion is not performed. This is of particular concern in young
children.
ONGOING CARE
Patients with VP or ventriculopleural shunts do not require prophylactic antibiotics for surgical or dental procedures in which the shunt is outside the
operative field. Prophylactic antibiotics may be helpful when the shunt is in the operative field, as described earlier, and should be routinely used
If a specimen of fluid is required from a peritoneal fluid collection, the tubing is then cut and aspirated distally. In a large pseudocyst, a liter or more of
fluid may be withdrawn. The distal catheter is then withdrawn from the patient and the catheter itself sent for culture. The International Medical University
remaining proximal tubing
should be observed to confirm CSF drainage and connected to a sterile, enclosed CSF drainage system. Patients with externalized shunts drain
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isotonic CSF and may suffer hyponatremia if appropriate fluid and electrolyte repletion is not performed. This is of particular concern in young
children.
ONGOING CARE
Patients with VP or ventriculopleural shunts do not require prophylactic antibiotics for surgical or dental procedures in which the shunt is outside the
operative field. Prophylactic antibiotics may be helpful when the shunt is in the operative field, as described earlier, and should be routinely used
during a planned CSF shunt revision. For patients with ventriculoatrial CSF shunts in place, an argument can be made for antibiotic prophylaxis prior
to procedures likely to result in bacteremia such as dental procedures. Dental practice guidelines suggest this also. American Heart Association
guidelines for endocarditis prophylaxis recommend amoxicillin 2 g 30-60 minutes before the procedure for adults and 50 mg/kg for pediatric patients.
Shunt independence has been reported for children with a prior history of shunt placement. In this situation, a child previously dependent on a CSF
shunt becomes once again able to drain CSF independently. This most commonly occurs when a shunt has gone without revision for many years or
decades. It is discovered most commonly when a shunt is disconnected in the absence of clinical symptoms. One series reported that 3% of children
with hydrocephalus became shunt independent later in life. However, in the setting of a shunt found to be fractured on x-ray without ventricular
enlargement or clinical symptoms of shunt failure, it has been observed that CSF can drain between the fractured catheter segments through the
tunnel of chronic scar tissue that forms around the catheter after shunt placement. Therefore, caution should be exercised in the presumption of
shunt independence unless it has been verified by invasive testing. Patients incidentally found to have broken shunt catheters may reasonably be
referred to a neurosurgeon for evaluation.
Shunted hydrocephalus during pregnancy frequently raises concerns over management of headaches during pregnancy, mechanism of delivery,
and the potential impact of hydrocephalus on the pregnancy outcome. Pregnancy, particularly in the third trimester, increases intra-abdominal
pressures. For patients with VP shunts, this can lead to a relative decrease in CSF shunt function. In case series of pregnant patients with
hydrocephalus, headaches are not uncommonly reported, particularly during the third trimester. These may herald shunt failure, the evaluation of
which may be complicated by a desire to avoid radiation during pregnancy. MRI may be a useful option in these circumstances. In the absence of
radiographic manifestations of shunt failure or additional clinical symptoms, safe observation of headaches has been successfully practiced, but
treatment decisions must be individualized, and other diagnoses, particularly eclampsia and preeclampsia, must be considered. There is little
evidence to suggest that the presence of a CSF shunt is a contraindication to labor and vaginal delivery. It has been argued that patients who are
suspected of being symptomatic from increased ICP may benefit from avoiding protracted labor, but no comparative studies exist to provide a clear
answer. Hydrocephalus and the presence of a CSF shunt do not, per se, appear to impact pregnancy outcome. However, recalling that hydrocephalus is
coincident with other diagnoses, such as epilepsy and myelomeningocele, that do have a significant impact on the potential for birth defects, the
importance of prenatal care should be emphasized by providers.
CENTRAL NERVOUS SYSTEM INFECTIONS

Kyle Wu, MD, and Timothy R. Smith, MD, PhD
BRAIN ABSCESS
Brain abscesses are an uncommon entity, with approximately 2000 cases reported in the United States annually. There is a higher incidence in
developing countries, and men are affected slightly more often than women. Classically, cerebral abscesses arise locally from otorhinolaryngeal
infections or hematogenously from distant infections, although opportunistic infections have become an important consideration upon initial
presentation as well. The pathogenic organisms most commonly implicated are of the Streptococcus family; Staphylococcus aureus, Klebsiella, and
anaerobes are also frequent. In immunocompromised patients, it is important to include Toxoplasma, Listeria, and Nocardia as possible infectious
agents, as well as fungal and mycobacterial pathogens.
A patient with a brain abscess can present with nonspecific symptoms. Headache, nausea, vomiting, and altered mental status can occur due to
increased ICP, whereas unilateral headache, seizures, and various focal neurologic deficits may be observed based on location of the mass lesion.
Fever and nuchal rigidity are also seen in many cases. Additional findings in the newborn patient may include cranial enlargement, meningeal signs,
irritability, and failure to thrive.
Common risk factors for brain abscess include sinus, inner ear, or dental infections. These sources usually lead to formation of frontal or temporal
lobe abscesses through contiguous spread. Hematogenous spread from intra-abdominal, pelvic, pulmonary, or cardiac seeding occurs most
commonly via the middle cerebral artery. This may lead to septic emboli causing pseudoaneurysm, abscess, or microembolic infarction at the gray-
white junction. Major risk factors for developing hematogenous infectious processes includes intravenous drug use, access and placement of long-
Chapter 38: Neurosurgery, Timothy R. Smith Pagedisease.
term intravenous hardware, chronic lung infections, pulmonary shunting, osteomyelitis, intracardiac hardware, and congenital cyanotic heart 89 / 93
Penetrating head trauma, including neurosurgery, may introduce a nidus for infection with delayed abscess formation. Parasitic infections, such as
cysticercosis or toxoplasmosis, should be considered in recent foreign travelers or in immunocompromised patients.
Fever and nuchal rigidity are also seen in many cases. Additional findings in the newborn patient may include cranial enlargement, meningeal signs,
irritability, and failure to thrive.
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Common risk factors for brain abscess include sinus, inner ear, or dental infections. These sources usually lead to formation of frontal or temporal
lobe abscesses through contiguous spread. Hematogenous spread from intra-abdominal, pelvic, pulmonary, or cardiac seeding occurs most
commonly via the middle cerebral artery. This may lead to septic emboli causing pseudoaneurysm, abscess, or microembolic infarction at the gray-
white junction. Major risk factors for developing hematogenous infectious processes includes intravenous drug use, access and placement of long-
term intravenous hardware, chronic lung infections, pulmonary shunting, osteomyelitis, intracardiac hardware, and congenital cyanotic heart disease.
Penetrating head trauma, including neurosurgery, may introduce a nidus for infection with delayed abscess formation. Parasitic infections, such as
cysticercosis or toxoplasmosis, should be considered in recent foreign travelers or in immunocompromised patients.
The differential diagnosis for brain abscess is broad, including stroke, TBI, intracranial hemorrhage, subdural empyema, hydrocephalus, dural sinus
thrombosis, meningitis, encephalitis, and tumor. Radiographically, abscesses appear most similar to tumor and can often be mistaken. All of these
conditions can present with headaches and altered mental status. In the initial evaluation of brain abscess, blood work that should be drawn includes
a white blood cell count, pan-cultures, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP); however, normal test results do not rule out
an infectious diagnosis. The key to diagnosing brain abscess is correlating the clinical scenario with an imaging study, such as contrast-enhanced CT or
contrasted MRI (the gold standard). The classic finding on CT or MRI is a well-circumscribed circular lesion with a strongly contrast-enhancing rim. CT is
typically the first study obtained on presentation, although MRI is the imaging modality of choice as it can provide greater anatomic detail. MRI
evaluation for brain abscess should always include DWIs, which can help differentiate between ring-enhancing lesions of infectious and neoplastic
origin, as abscesses typically exhibit diffusion restriction, whereas neoplastic lesions generally do not.
Lumbar puncture is not recommended in instances of intracranial abscess because CSF results are often nondiagnostic, and lumbar puncture is
associated with a worsened outcome in patients with brain abscesses. Less than one quarter of patients have positive CSF cultures, and with a large
enough abscess, there is risk for transtentorial or brainstem herniation. CSF sampling should be considered only if parasitic pathogens are suspected
or to guide treatment in clear cases of CSF involvement with an unknown infectious source. A definitive diagnosis is made by biopsy sampling of the
abscess through surgical means.
The treatment of brain abscesses can involve medical therapy, surgical intervention, or both. Treatment should incorporate correcting the primary
source of infection (eg, treating a cardiac valvular vegetation or repairing a correctable heart defect). Initial surgical treatment may consist of needle
aspiration of the abscess, especially if in a deep or difficult-to-access location. The abscess can be monitored serially with imaging, and the procedure
repeated as necessary. This reduces the size of the lesion and may provide microbiologic information for tailoring antibiotic treatment. Total excision
via a craniotomy may be preferred if the abscess contains multiple loculations or is resistant to antibiotic therapy. Abscesses located near the cerebral
ventricles pose a unique problem, as surgical manipulation may introduce infection into the intraventricular space; however, if left uncontrolled, the
abscess itself may rupture into the ventricle and seed the CSF. Generally, if the abscess is periventricular, small, and appears stable over serial imaging,
it may be managed expectantly with medical therapy. If surgical management for cerebral abscess is chosen, it is advisable to perform surgery before
starting antibiotics in medically stable patients to allow for identification of organisms and their antibiotic sensitivities. Antibiotic therapy typically
consists of 6-8 weeks of intravenous treatment followed by 4-8 weeks of oral antibiotic medications. Patients should receive routine follow-up imaging
and should also be initiated on an antiepileptic medication, which is often continued beyond the initial period of therapy. Glucocorticoids may be
considered to mediate symptomatic intracranial hypertension caused by significant vasogenic edema, although there is mixed evidence on the use of
steroids, and definitive treatment through mass reduction is favored.
In certain situations, medical therapy may suffice without the need for surgery. These situations include an abscess in its early stages (ie, symptoms for
< 2 weeks), a small (< 3 cm) abscess, or definite clinical improvement after 1 week of antibiotic therapy. Medical treatment without surgery should also
be considered in poor surgical candidates, patients with multiple abscesses and/or concomitant meningitis, patients with abscesses in eloquent
locations, or patients with hydrocephalus and ventricular shunts.
Patients with brain abscess have a reported mortality risk of 0%-30% depending on etiology and presentation. An overall 50% morbidity risk of
permanent neurologic deficits is conferred, which depends heavily on the severity of presenting symptoms.
SUBDURAL EMPYEMA
A subdural empyema is a collection of pus that forms in the subdural space. It is less common than brain abscess, but like abscesses, it is more
commonly found in males. Subdural empyema is an especially challenging condition because, unlike the brain parenchyma, the subdural space does
not pose much of a barrier to prevent the spread of infection. Additionally, antibiotics have poor penetration into the subdural space.
The most common cause of subdural empyema (70%) is contiguous spread from paranasal sinusitis, especially in cases involving the frontal sinus.
Chronic otitis media accounts for another 15% of cases. Osteomyelitis of the skull can also result in direct bacterial invasion of the brain and meninges.
As such, the organisms typically cultured from a subdural empyema include Streptococcus (aerobic and anaerobic) and Staphylococcus. Symptoms
experienced by patients with subdural empyema include fever, headache, nuchal rigidity, hemiparesis, and altered mental status. Other common Page 90 / 93
symptoms include seizures and sinus tenderness.
CT or MRI imaging will typically diagnose a subdural empyema. Three-fourths of empyemas are located over the convexity, whereas 15% are
A subdural empyema is a collection of pus that forms in the subdural space. It is less common than brain abscess, but like abscesses, it is more
commonly found in males. Subdural empyema is an especially challenging condition because, unlike the brain parenchyma, the subdural space does
not pose much of a barrier to prevent the spread of infection. Additionally, antibiotics have poor penetration into the subdural space.
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The most common cause of subdural empyema (70%) is contiguous spread from paranasal sinusitis, especially in cases involving the frontal sinus.
Chronic otitis media accounts for another 15% of cases. Osteomyelitis of the skull can also result in direct bacterial invasion of the brain and meninges.
As such, the organisms typically cultured from a subdural empyema include Streptococcus (aerobic and anaerobic) and Staphylococcus. Symptoms
experienced by patients with subdural empyema include fever, headache, nuchal rigidity, hemiparesis, and altered mental status. Other common
symptoms include seizures and sinus tenderness.
CT or MRI imaging will typically diagnose a subdural empyema. Three-fourths of empyemas are located over the convexity, whereas 15% are
parafalcine (ie, adjacent to the falx cerebri). Just as with brain abscesses, lumbar puncture should be avoided due to the potential risk of herniation,
and generally low culture yield.
The majority of surgical cases of subdural empyema require drainage for removal of mass effect and microbiologic reduction. The two primary surgical
options are burr-hole drainage and craniotomy. Although burr-hole drainage is less invasive, it is also less effective in cases of loculated or adherent
collections; thus, craniotomy is generally the preferred surgical option. Antibiotics are used for a course of 4-6 weeks, and patients are initiated on
therapeutic or prophylactic antiseizure medication. Medical treatment alone can be effective if the empyema is small, there is minimal neurologic
involvement, and early antibiotics demonstrate efficacy.
Subdural empyema carries a 15% mortality rate. Half of patients will have residual neurologic deficits at the time of hospital discharge. Factors known
to be associated with poor prognosis include age greater than 60 years, obtunded or comatose state at presentation, and empyema formation
secondary to surgery or trauma.
OSTEOMYELITIS
Osteomyelitis can affect the skull or the spinal vertebrae. Osteomyelitis of the skull usually results by contiguous spread from an infected sinus or from
penetrating trauma (eg, postoperative). The infectious agents are typically S aureus or Staphylococcus epidermidis, and treatment consists primarily
of prolonged antibiotic therapy (intravenously for the first 1-2 weeks). If resistant to treatment or the patient develops continued spread of the
infection, surgical treatment aimed at removing the infected bone may be required. Cranioplasty or other hardware placement is generally avoided
until several months later in order to minimize the risk of reestablishing the infection by providing an avascular nidus.
Vertebral osteomyelitis (VO) represents 3% of all cases of osteomyelitis and is more common than cranial osteomyelitis because of the spine’s rich
vascular supply. Both anterograde arterial seeding as well as retrograde venous plexus spread have been implicated in VO, with S aureus being the
most common organism. VO caused by Mycobacterium tuberculosis is known as Pott disease. Those at higher risk for developing VO include diabetics,
intravenous drug users, immunosuppressed patients, patients on hemodialysis, and the elderly.
The most common presentation in patients with VO is that of back pain (> 90%), usually unaffected by activity. Other typical presenting symptoms
include fever, weight loss, radicular pain, and myelopathy. Neurologic symptoms are usually a result of spread to the epidural space causing epidural
spinal abscess or from destruction of the vertebral body leading to collapse of the neural foramen, which results in nerve root compression. The most
commonly affected segment of the spine is the lumbar region followed by the thoracic, cervical, and sacral segments, respectively. Any source of
infection can theoretically put one at risk for developing VO, although important sources include infections of the urinary tract, cardiopulmonary
system, and mouth. VO also develops at sites of prior spine surgery.
Definitive diagnosis of VO is made with positive cultures, either from biopsy of the tissue itself or via blood cultures in the setting of suggestive
radiographic findings. Contrast-enhanced MRI has demonstrated excellent diagnostic sensitivity and is the preferred diagnostic modality. When
unable to obtain MRI, bone scintigraphy with SPECT is also highly effective in detection of VO.
The treatment of VO is nonsurgical in the vast majority of cases, with disease resolution being accomplished via antibiotic therapy alone. The goal of
therapy should be to minimize neurologic involvement and to maintain structural stability of the spine. Surgical treatment is considered to obtain a
tissue diagnosis if both blood cultures and closed-needle biopsy are nondiagnostic. In patients with a worsening neurologic deficit, the onset of
structural instability, or a failure of medical management, surgery is warranted for abscess drainage, alleviation of compression, and stabilization.
SPINAL EPIDURAL ABSCESS
Spinal epidural abscesses (SEAs) are often associated with VO, with the microbiologic isolate being S aureus in the majority of cases. The
Streptococcus species are the second most commonly implicated organism (Figure 38–27). SEAs are located most often in the thoracic region (50%),
followed by the lumbar (35%) and cervical (15%) regions. The vast majority of abscesses (80%) are located posterior to the spinal cord.
Figure 38–27.
Sagittal magnetic resonance T1-weighted image with contrast of the lumbar spine demonstrating diskitis/osteomyelitis associated with a spinal
epidural abscess.
SPINAL EPIDURAL ABSCESS
Spinal epidural abscesses (SEAs) are often associated with VO, with the microbiologic isolate being S aureus in the majority of cases. The
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Streptococcus species are the second most commonly implicated organism (Figure 38–27). SEAs are located most often in the thoracic region (50%),
followed by the lumbar (35%) and cervical (15%) regions. The vast majority of abscesses (80%) are located posterior to the spinal cord.
Figure 38–27.
Sagittal magnetic resonance T1-weighted image with contrast of the lumbar spine demonstrating diskitis/osteomyelitis associated with a spinal
epidural abscess.
The primary infection leading to SEA can be from hematogenous spread or direct extension. Hematogenous spread is more common, with skin
infections being the most common originating source. Other sources of hematogenous spread include nonsterile intravenous injections, bacterial
endocarditis, urinary tract infections, respiratory infections, and oropharyngeal abscesses. SEAs caused by direct extension can be from decubitus
ulcers or penetrating trauma, including after spinal procedures.
Patients with SEA are typically middle-aged. Risk factors for developing SEA include diabetes, peripheral vascular disease, intravenous drug use,
chronic renal failure, and alcoholism. Patients often present with back pain, spine tenderness, fever, sweats, and rigors. When motor weakness
ensues, there is a very rapid progression to paraplegia and bowel or bladder dysfunction. Thus, the diagnosis and treatment of SEA are emergent for
preservation and reversal of neurologic injury.
The workup of spinal epidural abscesses should include a complete blood count, ESR, CRP, and cultures. A lumbar puncture is contraindicated given
the potential to spread infection from the epidural to the intradural space. The imaging modality of choice is gadolinium-enhanced MRI, although both
contrast CT and myelography (although never used in lumbar SEA for risk of intradural seeding) may also be used to arrive at a diagnosis when the use
of MRI is precluded. Standing scoliosis x-rays (ie, 36-inch long cassette stand x-rays) may be used to follow patients at high risk of developing spinal
deformity (ie, kyphotic deformity).
The treatment of SEA, as with most infections of the CNS, is a combination of medical therapy plus surgery if needed. In patients who have small spinal
epidural abscesses without neurologic deficit, primary management with antibiotics alone is often sufficient. Emergent decompressive surgery via
laminectomy and washout is required for patients with new neurologic dysfunction. Surgery may be required on a subacute basis to drain pus and
debride granulation tissue if the infection remains treatment resistant. In cases where infection leads to bony instability, most often due to VO, internal
fixation with spinal hardware may be necessary, most often following resolution of the acute phase of the infection. Antibiotics are often given
intravenously for 4-6 weeks and then orally for another 4 weeks. The overall prognosis for patients with SEA is relatively poor, with a mortality rate of
20%. In patients who survive, restoration of baseline neurologic function is relatively rare.
Arko LT, Quach E, Nguyen V, et al. Medical and surgical management of spinal epidural abscess: a systematic review. Neurosurg Focus.
2014;37(2):E4. [PubMed: 25081964]
Berbari EF, Steckelberg JM, Osmon DR: Osteomyelitis. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and
Practice of Infectious Diseases, Updated Edition . 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 106.
Boody BS, Jenkins TJ, Maslak J, et al: Vertebral osteomyelitis and spinal epidural abscess: an evidence-based review. J Spinal Disord Tech.
2015;28(6):E316–E327. [PubMed: 26079841]
Darouiche RO: Spinal epidural abscess. N Engl J Med. 2006;355(19):2012–2020. [PubMed: 17093252]
Kusuma S, Klineberg EO: Spinal infections: diagnosis and treatment of discitis, osteomyelitis, and epidural abscess. In: Steinmetz MP, Benzel EC, eds.
2014;37(2):E4. [PubMed: 25081964]
Access Provided by:
Berbari EF, Steckelberg JM, Osmon DR: Osteomyelitis. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and
Practice of Infectious Diseases, Updated Edition . 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 106.
Boody BS, Jenkins TJ, Maslak J, et al: Vertebral osteomyelitis and spinal epidural abscess: an evidence-based review. J Spinal Disord Tech.
2015;28(6):E316–E327. [PubMed: 26079841]
Darouiche RO: Spinal epidural abscess. N Engl J Med. 2006;355(19):2012–2020. [PubMed: 17093252]
Kusuma S, Klineberg EO: Spinal infections: diagnosis and treatment of discitis, osteomyelitis, and epidural abscess. In: Steinmetz MP, Benzel EC, eds.
Benzel’s Spine Surgery . 4th ed. Philadelphia, PA: Elsevier; 2017:chap 122.

Chapter 38 - Neurosurgery

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Chapter 38 - Neurosurgery

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Current Diagnosis & Treatment: Surgery, 15e

Chapter 38: Neurosurgery

DIAGNOSIS & MANAGEMENT OF ALTERATIONS IN CONSCIOUSNESS

PATHOPHYSIOLOGY OF ALTERED CONSCIOUSNESS

PATHOPHYSIOLOGY OF ELEVATED INTRACRANIAL PRESSURE

SIGNS & SYMPTOMS OF INCREASED ICP

Cerebral Herniation Syndromes

Cerebellar Tonsillar Herniation

Upward Transtentorial Herniation

DIAGNOSTIC EVALUATION OF ALTERED CONSCIOUSNESS

Best motor response:

Abnormal flexion posturing

Best motor response:

Abnormal flexion posturing 3

MANAGEMENT OF ALTERED CONSCIOUSNESS

GENERAL MANAGEMENT OF ELEVATED ICP

1. Blood pressure control

11. Decompressive craniectomy

2007;24:S37–S44. [PubMed: 17511544]

IMAGING OF THE CENTRAL NERVOUS SYSTEM

BASIC CENTRAL NERVOUS SYSTEM IMAGING TECHNIQUES

5. MRI is an imaging technique that does not use ionizing radiation.

ADVANCED CENTRAL NERVOUS SYSTEM APPLICATIONS OF MRI, CT, AND ANGIOGRAPHY

Magnetic Resonance Imaging

B. Diffusion MRI and Perfusion MRI

B. Diffusion MRI and Perfusion MRI

A. Endovascular Coiling of Aneurysms

E. Treating Intraluminal Thrombus

The Glasgow Coma Scale

GCS 9-13 = moderate

GCS 14-15 = mild

TYPES AND MANAGEMENT OF PRIMARY TRAUMATIC BRAIN INJURY

Penetrating Injury & Gunshot Wounds

Acute epidural hematoma after head trauma in a 5-year-old patient.

B. Acute Subdural Hematoma

Diffuse Axonal Injury

TYPES AND MANAGEMENT OF SECONDARY TRAUMATIC BRAIN INJURY

Physiology of Increased Intracranial Pressure

CPP = MAP – ICP

Management of Increased Intracranial Hypertension

Management of Increased Intracranial Hypertension

A. Optimization of Cerebral Venous Outflow

H. Fever and Hypothermia

H. Fever and Hypothermia

J. Surgical Decompressive Craniectomy

CLINICAL OUTCOMES AFTER TRAUMATIC BRAIN INJURY

SPINAL CORD INJURY

A. Central Cord Syndrome

B. Anterior Cord Syndrome

D. Conus Medullaris Syndrome

Prognosis & Outcome

PERIPHERAL NERVE LESIONS

ACUTE PERIPHERAL NERVE INJURIES

Treatment & Prognosis

PERIPHERAL ENTRAPMENT NEUROPATHIES

Treatment & Prognosis

PERIPHERAL NERVE TUMORS

Treatment & Prognosis

Frontal lobe1 Impaired intellectual function

Dominant temporal lobe Aphasia

Nondominant temporal lobe Seizures

Uncus CN III palsy