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HydrocephalusPediatricsMerckManualsProfessionalEdition

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Hydrocephalus
By
Stephen J. Falchek, MD
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Patient Education
Hydrocephalus is accumulation of excessive amounts of CSF, causing cerebral ventricular
enlargement and/or increased intracranial pressure. Manifestations can include enlarged
head, bulging fontanelle, irritability, lethargy, vomiting, and seizures. Diagnosis is by
ultrasonography in neonates and young infants with an open fontanelle and by CT or MRI in
older infants and children. Treatment ranges from observation to surgical intervention,
depending on severity and progression of symptoms.
Hydrocephalus is the most common cause of abnormally large heads in neonates.
Hydrocephalus that develops only after the fontanelles have closed does not increase head
circumference or cause the fontanelle to bulge but can markedly and rapidly increase
intracranial pressure.

Hydrocephalus (Enlarged Skull)

STEVE ALLEN/SCIENCE PHOTO LIBRARY

Etiology
Hydrocephalus can result from

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Obstruction of CSF ow (obstructive hydrocephalus)

Impaired resorption of CSF (communicating hydrocephalus)
It can be either congenital or acquired from events during or after birth.
Obstruction most often occurs in the aqueduct of Sylvius but sometimes at the outlets of the
4th ventricle (Luschka and Magendie foramina). The most common causes of obstructive
hydrocephalus are
Aqueductal stenosis
Dandy-Walker malformation
Chiari II type malformation
Aqueductal stenosis is narrowing of the outow pathway for CSF from the 3rd ventricle to the
4th ventricle. It may be either primary, or secondary to scarring or narrowing of the aqueduct
resulting from a tumor, hemorrhage, or infection. Primary aqueductal stenosis may involve
true stenosis (forking of the aqueduct into smaller, poorly functioning channels) or presence
of a septum in the aqueduct. Primary aqueductal stenosis may be inheritable; there are many
genetic syndromes, some which are x-linked (thus male infants inherit the condition from
otherwise unaffected mothers).
Dandy-Walker malformation is progressive cystic enlargement of the 4th ventricle.
In Chiari II type (formerly Arnold-Chiari) malformation, hydrocephalus occurs with spina bida
(see Spina Bida) and syringomyelia (see Syrinx of the Spinal Cord or Brain Stem). Signicant
elongation of the cerebellar tonsils in Chiari type I or midline vermis in Chiari type II causes
them to protrude through the foramen magnum, with beaking of the colliculi and thickening
of the upper cervical spinal cord.
Impaired resorption in the subarachnoid spaces usually results from meningeal
inammation, secondary either to infection or to blood in the subarachnoid space, resulting
from either subarachnoid or intraventricular hemorrhages, which are complications of
delivery, particularly in premature infants (see Intracranial Hemorrhage).

Symptoms and Signs

Neurologic ndings depend on whether intracranial pressure is increased, symptoms of which
in infants include irritability, high-pitched cry, vomiting, lethargy, strabismus, and bulging
fontanelle. Older, verbal children may complain of headache, decreased vision, or both.
Papilledema is a late sign of increased intracranial pressure; its initial absence does not
exclude hydrocephalus.
Consequences of chronic hydrocephalus may include precocious puberty in girls, learning
disorders (eg, diculties with attention, information processing, and memory), loss of vision,
and impaired executive function (eg, problems with conceptualizing, abstracting, generalizing,
reasoning, and organizing and planning information for problem-solving).
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Diagnosis
Prenatal ultrasonography
Neonates: Cranial ultrasonography
Older infants and children: CT or MRI
Diagnosis is often made by routine prenatal ultrasonography. After birth, diagnosis is
suspected if routine examination reveals an increased head circumference; infants may have
a bulging fontanelle or widely separated cranial sutures. Similar ndings can result from
intracranial, space-occupying lesions (eg, subdural hematomas, porencephalic cysts, tumors).
Macrocephaly may result from an underlying brain problem (eg, Alexander disease or
Canavan disease), or it may be a benign, sometimes inherited, feature characterized by an
increased amount of CSF surrounding a normal brain. Children suspected of having
hydrocephalus require cranial imaging by CT, MRI, or ultrasonography (if the anterior
fontanelle is open). Cranial CT or ultrasonography is used to monitor progression of
hydrocephalus once an anatomic diagnosis has been made. If seizures occur, an EEG may be
helpful.

Treatment
Sometimes observation or serial lumbar punctures
For severe cases, a ventricular shunt procedure
Treatment depends on etiology, severity, and whether hydrocephalus is progressive (ie, size of
the ventricles increases over time relative to the size of the brain). Mild, nonprogressive cases
may be observed with serial imaging studies and measurement of head size. To temporarily
reduce CSF pressure in infants, ventricular taps or serial lumbar punctures (if the
hydrocephalus is communicating) may be used.
Progressive hydrocephalus usually requires a ventricular shunt. Shunts typically connect the
right lateral ventricle to the peritoneal cavity or, rarely, to the right atrium via a plastic tube
with a one-way, pressure-relief valve. When a shunt is rst placed in an infant or older child
whose fontanelle is closed, rapid withdrawal of uid can cause subdural bleeding as the brain
shrinks away from the skull. When the fontanelles are open, the skull can decrease in
circumference to match the decrease in brain size; thus, some clinicians recommend an early
decision regarding shunt placement so that it can be done before fontanelle closure.
In a third ventriculostomy, an opening is created endoscopically between the 3rd ventricle and
the subarachnoid space, allowing CSF to drain. This procedure is often combined with ablation
of the choroid plexus and is becoming more commonly used in the US. It is particularly useful
in less developed countries where access to consistent neurosurgical care is often limited. In
certain cases (eg, hydrocephalus caused by primary aqueductal stenosis), third
ventriculostomy may be adequate primary treatment.

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A ventricular shunt that goes to the subgaleal space may be used in infants as a temporary
measure for patients who may not require a more permanent shunt.
Although some children do not need the shunt as they age, shunts are rarely removed
because of the risk of bleeding and trauma. Fetal surgery to treat congenital hydrocephalus
has not been successful.

Shunt complications
The type of ventricular shunt used depends on the neurosurgeons experience, although
ventriculoperitoneal shunts cause fewer complications than ventriculoatrial shunts. Shunt
complications include
Infection
Malfunction
Any shunt has a risk of infection. Manifestations include chronic fever, lethargy, irritability,
headache, or a combination and other symptoms and signs of increased intracranial pressure;
sometimes redness becomes apparent over the shunt tubing. Antibiotics effective against the
organism infecting the shunt, which may include skin ora, are given, and typically the shunt
must be removed and replaced.
Shunts can malfunction because of a mechanical obstruction (typically blockage at the
ventricular end) or because of fracture of the tubing. In either case, intracranial pressure can
increase, which, if sudden, can be a medical emergency. Children present with headache,
vomiting, lethargy, irritability, esotropia, or paralysis of upward gaze. Seizures may occur. If the
obstruction is gradual, more subtle symptoms and signs can occur, such as irritability, poor
school performance, and lethargy, which may be mistaken for depression. To assess shunt
function, a shunt series (x-rays of the shunt tubing) and neuroimaging studies are done. The
ability to compress the bulb that is present on many shunt systems is not a reliable sign of
shunt function.
After the shunt is placed, head circumference and development are assessed, and imaging is
done periodically.

Key Points

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Hydrocephalus is usually caused by obstruction to the normal ow of CSF but can be due
to impaired resorption of CSF.
If the disorder occurs before the cranial sutures have fused, the head may be enlarged,
with bulging fontanelles.
Neurologic symptoms develop mainly if intracranial pressure increases; infants may
have irritability, high-pitched cry, vomiting, lethargy, and strabismus.
Diagnose using ultrasonography prenatally and in neonates; use MRI or CT for older
children.
Treat with observation or serial lumbar punctures or a ventricular shunt procedure
depending on the etiology and severity and progression of symptoms.
Last full review/revision December 2014 by Stephen J. Falchek, MD
2016 Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA

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