Hydrocephalus

Dr. Deepa Khanal

3rd year resident
Pediatrics
KMCTH
 Hydrocephalus
• Term “hydrocephalus” is derived from Greek words
“hydro” = water; “cephalus” = head

• “Hydrocephalus” in Greek means “watery head”

• As the name implies, it is a condition in which there is

excessive accumulation of fluid in the brain

• Although once known as “water in the brain,” the “water” is

the “cerebrospinal fluid”
The ventricular system of brain

It consists of:
1. Two lateral ventricles
2. The third ventricle
3. Aqueduct of Sylvius
(Cerebral Aqueduct)
4. Fourth Ventricle `
 PHYSIOLOGY

• CSF is formed primarily in the ventricular system by the

choroid plexus of lateral, third & fourth ventricles.

• This is regulated actively by adrenergic and cholinergic nerves

that innervate the choroid plexus

• In a normal child, approx. 20 mL/hr of CSF is produced

• Total volume of CSF –

50 mL in an infant
150 mL in an adult
• CSF flow results from the pressure gradient between the
ventricular system and venous channels.

• Intraventricular pressure(180 mm H2O), whereas the pressure

in the superior sagittal sinus is 90 mm H2O
 Types of hydrocephalus

• The two major types:

– communicating hydrocephalus
– non-communicating (or obstructive) hydrocephalus
 Communicating hydrocephalus
• Communicating hydrocephalus occurs when the spinal fluid circulates freely
between the brain (where the CSF is produced) and the spaces outside the
brain- the subarachnoid space
• In communicating hydrocephalus, the obstruction of CSF flow is in the
subarachnoid space from prior bleeding or meningitis.
• This causes thickening of the arachnoids leading to blockage of the return-flow
channels.
• In some patients, the spaces filled by CSF are uniformly enlarged without an
increase in intracranial pressure.
• This special form of communicating hydrocephalus is called normal pressure
hydrocephalus, which results specifically from impaired CSF reabsorption at
the arachnoids granulations
 Non-Communicating Hydrocephalus
• In non-communicating hydrocephalus, the CSF in the ventricles can not
reach the subarachnoid space.

• This results from obstruction of:

– interventricular foramens
– cerebral aqueduct
– outflow foramens of the fourth ventricle (median and lateral apertures)

• The most common obstruction is in the cerebral aqueduct.

• A block at any of these sites leads rapidly to dilatation of one or more
ventricles.
• If the skull is still pliable as it is children younger than 2 years, the head may
enlarge
 A hydrocephalus B normal cerebrospinal fluid level

1 cerebrospinal fluid

2 ventricles

HYDROCEPHALUS NORMAL
 Causes of Hydrocephalus

COMMUNICATING NONCOMMUNICATING
Achondroplasia Aqueductal stenosis
Basilar impression Infectious*
Choroid plexus papilloma X-linked
Meningeal malignancy Mitochondrial
Meningitis Autosomal recessive
Posthemorrhagic Autosomal dominant
Benign enlargement of subarachnoid space Chiari malformation
Dandy-Walker malformation
Klippel-Feil syndrome
HYDRANENCEPHALY Mass lesions
Abscess
Holoprosencephaly Hematoma
Massive hydrocephalus Neurocutaneous disorders
Porencephaly Vein of Galen malformation
Walker-Warburg syndrome
 Congenital and Acquired Causes

• Congenital causes in infants and children:

– Brainstem malformation causing stenosis of the aqueduct of

Sylvius: This is responsible for 10% of all cases of hydrocephalus
in newborns.
– Dandy-Walker malformation: This affects 2-4% of newborns with
hydrocephalus.
– Arnold-Chiari malformation type 1 and type 2
– Agenesis of the foramen of Monro
– Congenital toxoplasmosis
– Bickers-Adams syndrome
• Acquired causes in infants and children:

– Mass lesions: Mass lesions account for 20% of all cases of hydrocephalus
in children. These are usually tumors (eg, medulloblastoma,
astrocytoma), but cysts, abscesses, or hematoma also can be the cause
– Hemorrhage: IVH can be related to prematurity, head injury, or rupture of
a vascular malformation.
– Infections: Meningitis (especially bacterial) and, in some geographic
areas, cysticercosis can cause hydrocephalus
– Increased venous sinus pressure: This can be related to Achondroplasia
– Iatrogenic: Hypervitaminosis A
– Idiopathic
• There are two other forms of hydrocephalus which do not fit
exactly into the categories mentioned above and primarily affect
adults:
– Hydrocephalus ex-vacuo
– Normal Pressure Hydrocephalus (NPH)
• Hydrocephalus ex-vacuo occurs when stroke or traumatic injury
cause damage to the brain
• In these cases, brain tissue may actually shrink
• Causes of NPH
(Most cases are idiopathic and are probably
related to a deficiency of arachnoid granulations):
– SAH
– Head trauma
– Meningitis
 Aqueductal stenosis
• One of the most common causes of hydrocephalus is “aqueductal
stenosis.”
• In this case, hydrocephalus results from a narrowing of the aqueduct of
Sylvius, a small passage between the third and fourth ventricles in the
middle of the brain.
 Normal pressure Hydrocephalus
• NPH is an abnormal increase of cerebrospinal fluid in the
brain’s ventricles that may result from a subarachnoid
hemorrhage, head trauma, infection, tumor, or complications
of surgery.
• However, many people develop NPH when none of these
factors are present
• An estimated 375,000 older Americans have NPH
 Dandy-Walker malformation
Cystic expansion of the fourth ventricle in the posterior fossa
and midline cerebellar hypoplasia, which results from a
developmental failure of the roof of the fourth ventricle
during embryogenesis.

90% of patients have hydrocephalus with agenesis of

the posterior cerebellar vermis and corpus callosum.
 Arnold-Chiari malformation
• Chiari malformations are characterized by cerebellar
elongation and protrusion through the foramen magnum into
the cervical spinal cord
• Primary anomalies of the hindbrain and skeletal structures
with consequent mechanical deformities produce four
different positions of the cerebellum and brainstem relative to
the foramen magnum and upper cervical canal
 Arnold-Chiari malformation

• Type I:
Produces symptoms during adolescence or adult life and is usually not
associated with hydrocephalus.
Recurrent headache, neck pain, urinary frequency, and progressive lower-
extremity spasticity

• The type II :
Progressive hydrocephalus with a myelomeningocele.
Approximately 10% of type II malformations produce symptoms durin
infancy, consisting of stridor, weak cry and apnea

• Chiari III and IV are very rare and may be life threatening
 Normal pressure hydrocephalus (NPH)
• The “normal pressure hydrocephalus” was described by Dr. Salomon
Hakim in 1964
• is an accumulation of cerebrospinal fluid that causes the ventricles in the
brain to become enlarged, sometimes with little or no increase in
intracranial pressure (ICP)
• It is most commonly seen in older adults, and is accompanied by some or
all of the following triad of symptoms:
1. Gait disturbances
2. Mild dementia
3. Impaired bladder control
 Clinical features of hydrocephalus
Influenced by:
• The age at onset
• The nature of the lesion causing obstruction,
• The duration and rate of increase of the
intracranial pressure (ICP).
Features of Increased Intracranial Pressure
In Infants In Children
Bulging fontanelle Diplopia
Failure to thrive Headache
Impaired upward gaze
Mental changes
(setting sun sign)
Large head Nausea and vomiting
Shrill cry Papilledema
Symptoms in infants include poor feeding, irritability, reduced activity, and
vomiting.
Symptoms in children and adults include the following:
• Slowing of mental capacity, cognitive deterioration
• Headaches
• Neck pain, suggesting tonsillar herniation
• Vomiting, more significant in the morning
• Blurred vision: A consequence of papilledema and, later, of optic atrophy
• Double vision: Related to unilateral or bilateral sixth nerve palsy
• Difficulty in walking secondary to spasticity
• Drowsiness
 Diagnosis
• Examination in infants may reveal the following findings:
– Head enlargement (head circumference ≥98th percentile
for age)
– Dysjunction of sutures
– Dilated scalp veins
– Tense fontanelle
– Setting-sun sign:
• Characteristic of increased intracranial pressure (ICP)
• downward deviation of ocular globes,
• retracted upper lids,
• visible white sclerae above iris
– Increased limb tone (spasticity preferentially affects the
lower limbs)
• Children and adults may demonstrate the following findings on
physical examination:
– Papilledema
– Failure of upward gaze: Due to pressure on the tectal plate
through the suprapineal recess; the limitation of upward
gaze is of supranuclear origin
– Unsteady gait
– Large head
– Unilateral or bilateral sixth nerve palsy (secondary to
increased ICP)
– Children may also exhibit the Macewen sign, in which a
"cracked pot" sound is noted on percussion of the head.
 Hydrocephalus seen on a
CT scan of the brain

Dilatation of lateral
ventricles-
Ventriculomegaly
 Hydranencephaly
• Hydranencephaly may be confused with hydrocephalus
• The cerebral hemispheres are absent or represented by membranous sacs
with remnants of frontal, temporal, or occipital cortex dispersed over the
membrane
• The midbrain and brainstem are relatively intact
• The cause of hydranencephaly is unknown, but bilateral occlusion of the
internal carotid arteries during early fetal development would explain most of
the pathologic abnormalities
• Affected infants can have a normal or enlarged head circumference at birth
that grows at an excessive rate postnatally
• Transillumination shows an absence of the cerebral hemispheres
• The child is irritable, feeds poorly, develops seizures and spastic quadriparesis,
and has little or no cognitive development.
• A ventriculoperitoneal shunt prevents massive enlargement of the cranium.
 Hydranencephaly

MRI scan showing the brainstem and spinal cord with remnants of the cerebellum and
the cerebral cortex. The remainder of the cranium is filled with cerebrospinal fluid
 Megalencephaly
• Megalencephaly is an anatomic disorder of brain growth defined as a
brain weight:volume ratio >98th percentile for age (or ≥2 standard
deviations above the mean) that is usually accompanied by macrocephaly
(an occipitofrontal circumference OFC>98th percentile).
• Various storage and degenerative diseases are associated with
megalencephaly, but anatomic and genetic causes exist as well
• The most common cause of anatomic megalencephaly is benign familial
megalencephaly.
• This condition is easily diagnosed by careful family history and
measurement of the parents’ head circumferences (OFCs).
• Anatomic megalencephaly is usually apparent at birth, and head growth
continues to run parallel to the upper percentiles.
• Sometimes, in some syndromes, increased OFC is the presenting sign.
• Neuroimaging is critical in identifying the various structural and gyral
abnormalities seen in syndromic macrocephaly and determining whether
anatomic megalencephaly exists.
• Common megalencephaly-associated macrocephaly syndromes include
syndromes with prenatal and/or postnatal somatic overgrowth such as
Sotos, Simpson-Golabi-Behmel, fragile X, Weaver, and Bannayan-
Ruvalcaba-Riley syndromes , and Gorlin syndromes.
• Sotos syndrome (cerebral gigantism) is the most common
megalencephalic syndrome, with 50% of patients having
prenatal macrocephaly and 100% of patients having
macrocephaly by age 1 yr.
• Early postnatal overgrowth normalizes by adulthood
• Facial features include high forehead with frontal bossing,
sparse hair in the frontoparietal region, downslanting
palpebral fissures, apparent hypertelorism, long narrow face,
prominent mandible, and malar flushing.
• Hypotonia, poor coordination, and speech delay are common.
• Most children show mental retardation, ranging from mild to
severe.
 Treatment

• Depends on the cause

• Medical management:
– Emergency management of acute increase of ICP
– b.    Slowly progressive hydrocephalus: Acetazolamide may be
effective in children age 2 weeks to 10 months with slowly
progressive communicating hydrocephalus.
• Use of acetazolamide and furosemide- provide temporary relief
by reducing the rate of CSF production
• Surgical management :
• Extracranial shunts: a ventriculoperitoneal shunt
• Endoscopic third ventriculostomy
Surgical: CSF shunting.  
– a.    Shunt types: Ventriculoperitoneal shunts used most
commonly. Ventriculoatrial and pleural shunts are
associated with cardiac arrhythmias, pleural effusions, and
higher rates of infection.   
– b.    Shunt complications: Shunt dysfunction may be caused
by infection, obstruction (clogging or kinking),
disconnection, and migration of proximal and distal tips.
Patient will develop signs of increased ICP with shunt
malfunction.
Ventriculoperitoneal shunt placement
 Prognosis
• They are at increased risk for various developmental disabilities

• The mean intelligence quotient is reduced particularly for

performance tasks

• Vision problems are common, including strabismus,

visuospatial abnormalities, visual field defects, optic atrophy
with decreased acuity

• Accelerated pubertal development- because of increased

gonadotropin secretion in response to increased ICP
Thank you