Syringomyelia

Background
Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord. Hydromyelia is a dilatation of
the central canal by cerebrospinal fluid (CSF) and may be included within the definition of syringomyelia. The
following are types of syringomyelia.

Syringomyelia with fourth ventricle communication

About 10% of syringomyelia cases are of this type. This communication can be observed on MRI. In some cases, a
blockage of CSF circulation occurs. A shunt operation may be the best therapeutic option for these patients.

Syringomyelia due to blockage of CSF circulation (without fourth

ventricular communication)
Representing at least 50% of all cases, this is the most common type of syringomyelia. Obstruction of CSF
circulation from the basal posterior fossa to the caudal space may cause syringomyelia of this type. The most
common example is Arnold-Chiari malformation, which is also associated with communicating syringomyelia. Other
causes include the following:

• Basal arachnoiditis (postinfectious, inflammatory, postirradiation, blood in subarachnoid space)[1]

• Basilar impression or invagination
• Meningeal carcinomatosis
• Pathological masses (arachnoid cysts, rheumatoid
• arthritis pannus, occipital encephalocele, tumors)

Syringomyelia due to spinal cord injury

Fewer than 10% of syringomyelia cases are of this type. Mechanisms of injury include (1) spinal trauma, (2)
radiation necrosis, (3) hemorrhage from aneurysm rupture or arteriovenous malformation or in a tumor bed, (4)
infection (spinal abscess, human immunodeficiency virus, transverse myelitis), and (5) cavitation following ischemic
injury or degenerative disease.

Syringomyelia and spinal dysraphism

Spinal dysraphism may cause syringomyelia through a variety of mechanisms, including those mentioned under the
previous three categories. Identification and treatment of associated dysraphism has the greatest impact on
arresting progression of syringomyelia.

Syringomyelia due to intramedullary tumors

Fluid accumulation is usually caused by secretion from neoplastic cells or hemorrhage. The tumors most often
associated with syringomyelia are ependymoma and hemangioblastoma. Extramedullary intradural and extradural
tumors are considered separately under the second category because the mechanism of syrinx formation is
blockage of the CSF pathway.

Idiopathic syringomyelia
Idiopathic syringomyelia has an unknown cause and cannot be classified under any of the previous categories.[2]
Surgical decompression can help in some patients with remarkable neurologic deficit.

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Pathophysiology
Although many mechanisms for syrinx formation have been postulated, the exact pathogenesis is still unknown.
Frequently cited theories are those of Gardner, William, and Oldfield.

Gardner's hydrodynamic theory

This theory proposes that syringomyelia results from a "water hammer"-like transmission of pulsatile CSF pressure
via a communication between the fourth ventricle and the central canal of the spinal cord through the obex. A
blockage of the foramen of Magendie initiates this process.[3]

William's theory
This theory proposes that syrinx development, particularly in patients with Chiari malformation, follows a differential
between intracranial pressure and spinal pressure caused by a valvelike action at the foramen magnum.[4] The
increase in subarachnoid fluid pressure from increased venous pressure during coughing or Valsalva maneuvers is
localized to the intracranial compartment.

The hindbrain malformation prevents the increased CSF pressure from dissipating caudally. During Valsalva, a
progressive increase in cisterna magna pressure occurs simultaneously with a decrease in spinal subarachnoid
pressure. This craniospinal pressure gradient draws CSF caudally into the syrinx.

Oldfield's theory
Downward movement of the cerebellar tonsils during systole can be visualized with dynamic MRI. This oscillation
creates a piston effect in the spinal subarachnoid space that acts on the surface of the spinal cord and forces CSF
through the perivascular and interstitial spaces into the syrinx raising intramedullary pressure. Signs and symptoms
of neurological dysfunction that appear with distension of the syrinx are due to compression of long tracts, neurons,
and microcirculation. Symptoms referable to raised intramedullary pressure are potentially reversible by syrinx
decompression.[5]

The intramedullary pulse pressure theory

The here-proposed intramedullary pulse pressure theory instead suggests that syringomyelia is caused by
increased pulse pressure in the spinal cord and that the syrinx consists of extracellular fluid. A new principle is
introduced implying that the distending force in the production of syringomyelia is a relative increase in pulse
pressure in the spinal cord compared to that in the nearby subarachnoid space. The formation of a syrinx then
occurs by the accumulation of extracellular fluid in the distended cord.

Etiology
Etiology of syringomyelia often is associated with craniovertebral junction abnormalities.

Bony abnormalities include the following:

• Small posterior fossa

• Platybasia and basilar invagination
• Assimilation of the atlas

Soft-tissue masses of abnormal nature include the following:

• Tumors (eg, meningioma at foramen magnum)

• Inflammatory masses

Neural tissue abnormalities include the following:

• Cerebellar tonsils and vermis herniation

• Chiari malformation

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Membranous abnormalities include the following:

• Arachnoid cysts[11] , rhombic roof, or vascularized membranes

• Posthemorrhagic or postinflammatory membranes

Other etiologies not associated with craniovertebral abnormalities may include the following:

• Arachnoid scarring related to spinal trauma

• Arachnoid scarring related to meningeal inflammation
• Arachnoid scarring related to surgical trauma
• Subarachnoid space stenosis due to spinal neoplasm or vascular malformation
• Subarachnoid space stenosis, with possible scarring, related to disk and osteophytic disease
• Idiopathic

Epidemiology
Estimated prevalence of the disease is about 8.4 cases per 100,000 people and occurs more frequently in men than
in women. The disease usually appears in the third or fourth decade of life, with a mean age of onset of 30 years.
Rarely, syringomyelia may develop in childhood or late adulthood.

Prognosis
Prognosis depends on the underlying cause, the magnitude of neurological dysfunction, and the location and
extension of the syrinx.

Patients presenting with moderate or severe neurological deficits fare much worse than those patients with mild
deficits. Patients with central cord syndrome have poor response to treatment.

Natural history of syringomyelia still is not well understood. Although older studies had suggested that 20% of
patients died at an average age of 47 years, mortality rates are likely lower in today's patients as a result of surgical
interventions and better treatment of complications associated with significant paresis, such as pulmonary
embolism.[15]

Complications
Myelopathy is the most serious consequence of syringomyelia. The following are the seven grade classifications of
disability from myelopathy according to the Modified Nurick Classification.

• Grade 0 - No root signs or symptoms

• Grade I - Root signs or symptoms; no evidence of cord involvement
• Grade II - Signs of cord involvement; normal gait
• Grade III - Mild gait abnormality; able to be employed
• Grade IV - Gait abnormality prevents employment
• Grade V - Able to ambulate only with assistance
• Grade VI - Chairbound or bedridden

Complications due to myelopathy include the following:

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 • Recurrent pneumonia
• Paraplegia or quadriplegia
• Decubitus ulcers
• Bowel and urinary dysfunction

Mortality/morbidity
Assessing treatment results is difficult because of the rarity of syringomyelia, variability of presentation and natural
history, and the relatively short follow-up in most studies.

In one study, half of all patients with syringomyelia were in clinically stable condition for several years.

Although an older study had suggested that 20% of patients died at an average of 47 years, mortality rates are likely
lower in today's patients as a result of surgical interventions and better treatment of complications associated with
significant paresis, such as pulmonary embolism.

History
Syringomyelia usually progresses slowly; the course may extend over many years. The condition may have a more
acute course, especially when the brain stem is affected (i.e., syringobulbia). Syringomyelia usually involves the
cervical area. Symptomatic presentation depends primarily on the location of the lesion within the neuraxis. Clinical
manifestations are discussed in the sections that follow.

Sensory
Syrinx interrupts the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in loss
of these sensations, while light touch, vibration, and position senses are preserved (dissociated sensory loss).

When the cavity enlarges to involve the posterior columns, position and vibration senses in the feet are lost;
astereognosis may be noted in the hands.

Pain and temperature sensation may be impaired in either or both arms, or in a shawllike distribution across the
shoulders and upper torso anteriorly and posteriorly.

Dysesthetic pain, a common complaint in syringomyelia, usually involves the neck and shoulders, but may follow a
radicular distribution in the arms or trunk. The discomfort, which is sometimes experienced early in the course of the
disease, generally is deep and aching and can be severe.

Motor
Syrinx extension into the anterior horns of the spinal cord damages motor neurons (lower motor neuron) and causes
diffuse muscle atrophy that begins in the hands and progresses proximally to include the forearms and shoulder
girdles. Clawhand may develop.

Respiratory insufficiency, which usually is related to changes in position, may occur.

Autonomic
Impaired bowel and bladder functions usually occur as a late manifestation.

Sexual dysfunction may develop in long-standing cases.

Horner syndrome may appear, reflecting damage to the sympathetic neurons in the intermediolateral cell column.

Extension of the syrinx

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A syrinx may extend into the medulla, producing a syringobulbia.[6, 7] This syndrome is characterized by dysphagia,
nystagmus, pharyngeal and palatal weakness, asymmetric weakness and atrophy of the tongue, and sensory loss
involving primarily pain and temperature senses in the distribution of the trigeminal nerve.

Rarely, the syrinx cavity can extend beyond the medulla in the brain stem into the centrum semiovale
(syringocephalus).

Lumbar syringomyelia can occur and is characterized by atrophy of the proximal and distal leg muscles with
dissociated sensory loss in the lumbar and sacral dermatomes. Lower limb reflexes are reduced or absent.
Impairment of sphincter function is common.

Other manifestations
Painless ulcers of the hands are frequent. Edema and hyperhidrosis can be due to interruption of central autonomic
pathways.

Neurogenic arthropathies (Charcot joints) may affect the shoulder, elbow, or wrist.[8] Scoliosis is seen sometimes.[9,
10]

Acute painful enlargement of the shoulder is associated with destruction of the head of the humerus.

Physical Examination
A complete physical examination may reveal diminished arm reflexes, which are sometimes present early in the
clinical course of syringomyelia.

Lower limb spasticity, which may be asymmetrical, appears with other long-tract signs such as paraparesis,
hyperreflexia, and extensor plantar responses.

Rectal examination includes an evaluation of volitional sphincter control and sensory assessment of sacral
dermatomes.

Dissociated sensory impairment may be noted.

The syrinx may extend into the brain stem, affecting cranial nerves or cerebellar function.

Brainstem signs are common in syringomyelia associated with Chiari malformations.

DDx
Diagnostic Considerations
Failure to recognize the symptoms of syringomyelia or attributing symptoms (pain, excess sweating, increased
spasticity, numbness, or weakness) to other causes can result in morbidity, including neurologic deterioration.
Clinicians should have a high index of suspicion for syringomyelia in any patient with SCI presenting with new onset
or worsening of any of the aforementioned symptoms.

The presence of a peripheral nerve disorder can alter the signs of myelopathy, masking both the sensory loss and
distal hyperreflexia. Worsening syringomyelia could be missed in this setting.

Differential Diagnoses
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 • Acute Inflammatory Demyelinating Polyradiculoneuropathy
• Amyotrophic Lateral Sclerosis in Physical Medicine and Rehabilitation
• Atlantoaxial Instability in Down Syndrome
• Brainstem Gliomas
• Central Pontine Myelinolysis
• Chronic Inflammatory Demyelinating Polyradiculoneuropathy
• Diabetic Neuropathy
• Diagnosis and Management of Cervical Spondylosis
• Hydrocephalus
• Imaging in Ankylosing Spondylitis
• Meningioma
• Motor Evoked Potentials
• Neural Tube Defects
• Neuro-Ophthalmologic Manifestations of Multiple Sclerosis (MS)
• Neurological History and Physical Examination
• Pediatric Ependymoma
• Pediatric Medulloblastoma
• Physical Medicine and Rehabilitation for Limb-Girdle Muscular Dystrophy
• Spinal Cord Hemorrhage
• Spinal Cord Infarction
• Spinal Cord Trauma and Related Diseases
• Spinal Epidural Abscess
• Spinal Metastasis
• Spinal Muscular Atrophy
• Vascular Surgery for Arteriovenous Malformations

Workup

Workup
Imaging Studies
The initial evaluation of patients suspected of having a spinal cord syrinx includes a comprehensive history and
physical examination.

Information obtained from examinations guides the imaging studies. Essential tests include plain radiographic series
with dynamic views and high-resolution CT scan to assess the bony spinal canal.

The most sensitive imaging test for soft tissue is an MRI scan. Gadolinium-enhanced images are also helpful in
differentiating between tumor, scar, and disk material, especially in postoperative or posttraumatic cases.

Magnetic resonance imaging

Physicians primarily use magnetic resonance imaging (MRI) to diagnose syringomyelia.

Imaging of the entire rostrocaudal extension of the cyst or cysts is important. Gadolinium-enhanced images are
indicated if a tumor is suspected. Gadolinium-enhanced images are helpful in differentiating between scar or disk
material associated with a syrinx, especially in postoperative or posttraumatic cases.

MRI examination should include sagittal and transverse views in T1 and T2 images (see image below). Proton
density scans also can be helpful.

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 Sagittal T1-weighted image showing a thoracic syrinx.

Magnetic resonance angiography can be especially helpful in cases of syringomyelia associated with vascular
lesions.

Cine phase-contrast MRI is used to analyze CSF flow dynamics near the spinal cord cyst.

Myelography
Myelography is performed in special situations when MRI cannot be used. Widening of the cord and complete
subarachnoid block may be observed.

Myelogram combined with immediate and delayed high-resolution CT scan also can be performed. Delayed CT
scans are obtained 4-24 hours after the initial testing and can demonstrate cyst filling.

Other Tests
In neurophysiological assessment by somatosensory evoked potentials (SSEPs), low-amplitude or delayed
responses are present in myelopathy.

Neurophysiological assessment by motor evoked response may be more sensitive than SSEPs in the evaluation of
spinal cord dysfunction.

Histologic Findings
The syringomyelic cavity, or syrinx, forms most commonly in the lower cervical region, particularly at the base of the
posterior horn and extending into the central gray matter and anterior commissure of the cord.

Histopathologic findings include (1) cavitation of spinal cord gray matter, (2) syrinx continuous with or adjacent to the
central canal, and (3) an inner layer of gliotic tissue.

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In association with the syrinx, other pathological conditions such as tumors, vascular anomalies, or infective
processes also may be evident.

Treatment
Medical Care
No medical treatment is known for patients with syringomyelia. However, a chronic, stable clinical course is
common. Identifying the underlying cause of syrinx formation is very important. Surgical treatment most likely will be
necessary.

Neurorehabilitative care facilitates preservation of remaining neurological functions and prevents complications of
quadriparesis such as infection and decubitus ulcers.

Surgical Care
A variety of surgical treatments have been proposed for syringomyelia and are discussed in the sections that follow.

Suboccipital and cervical decompression

This operation includes suboccipital craniectomy; laminectomy of C1, C2, and sometimes C3; and duraplasty.[12,
13]

Some authors report microsurgical lysis of any adhesions, opening of the fourth ventricular outlet, and plugging of
the obex (later steps are based on Gardner's hydrodynamic theory).

Laminectomy and syringotomy (dorsolateral myelotomy)

After decompression, the syrinx is drained into the subarachnoid space through a longitudinal incision in the dorsal
root entry zone (between the lateral and posterior columns), usually at the level of C2-C3.

Incision in the dorsal root entry area has the minimum risk of increasing neurological deficit.

Shunts
The following types of shunts may be indicated:

• Ventriculoperitoneal shunt - Indicated if ventriculomegaly and increased intracranial pressure are present
• Lumboperitoneal shunt - Placed infrequently because of increased risk of herniation through the foramen
magnum
• Syringosubarachnoid dorsal root entry zone shunt
• Syringoperitoneal shunt

Percutaneous needling
This technique is advocated as a possible mode of therapy; however, rapid refilling of the hydromyelic cavity from
the ventricular system follows aspiration of fluid at the time of surgery. Moreover, a needle track seems unlikely to
remain open.

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Terminal ventriculostomy
The terminal ventricle is the dilated portion of the central canal that extends below the tip of the conus medullaris
into the filum terminale. A laminectomy is performed over the caudal limit of the fluid sac, and the filum is opened.

This procedure is suitable only in patients with symptoms of syrinx without Chiari malformation. It is inappropriate in
cases in which the hydromyelic cavity does not extend into the lumbar portion of the spinal cord or into the filum
terminale.

Neuroendoscopic surgery
This technique is particularly useful in evaluating and treating multiple septate syrinxes.

A fibroscope inserted through a small myelotomy allows inspection of the intramedullary cavity. Septa are
fenestrated, either mechanically or by laser. Fluid from the cavity is then shunted into the subarachnoid space.

Surgical untethering in select cases with posttraumatic tethering associated with syringomyelia[14]