Hydrocephalus

1. Amaan Mohiuddin
Gary Oh

3. INTRODUCTION
 Hydrocephalus translates to "water on the brain“
 Disease or anatomical defect causes an increase in the
amount of cerebrospinal fluid (CSF) present in the
cranium results in increased pressure against the brain
tissue
 Caused by a variety of disorders and disease states,
thus making it complex to define and diagnose

4. EPIDEMIOLOGY
 Incidence of congenital hydrocephalus 2-3/1000 live
births
 Incidence equal in males and females
 15-25% of neonates with open myelomeningocele (a
form of spina bifida)
 Patients with myelomeningocele who require shunting
reaches 80-90%
 56,600 children and adolescents younger than age 18
years have a shunt in place

5. PATHOPHYSIOLOGY
 CSF is produced by the choroid plexus in the third and
fourth ventricles in the brain.
 Choroid plexus (cerebral ventricles). Consists of villous
folds lined by epithelium with a central core of highly
vascularized connective tissue. Secretion and diffusion
 CSF fills the subarachnoid spaces, protecting and
cushioning the brain
 Volume - 50 mL (infants) & 150 mL (adults)
 25 percent is within the ventricular system.
 CSF formation continues in raised intracranial pressure
unless extremely high

6.  When hydrocephalus occurs, part of this process is
blocked, although different parts are blocked depending
upon the type of hydrocephalus.
 Infantile hydrocephalus most associated with the
congenital anomalies including spina bifida and
aqueductal stenosis.
 Spina bifida is a midline defect in the mesenchymal-
derived tissues and is classified as either a closed or open
neural tube defect (NTD)
 Most common cause of congenital hydrocephalus is
obstruction of the cerebral aqueduct — the passageway
between the third and fourth ventricle of the brain

11. CAUSES, DIFFERENTIAL DX
Inherited
 Myelodysplasia
 Atresia of formen of Monro
 NT defect
 Encephalocele
 Spina bifida
 Genetic abnormalities
 Aqueductal stenosis
 Dandy-Walker syndrome
 Other cerebral and spinal
malformations
Acquired
 Tumor
 Meningitis
 Infection
 Hemorrhage
 Arachnoid Cyst
 Posterior Fossa Cyst
 TBI
 Idiopathic

12.  Chiari malformations, an abnormality at the base of brain
where the spinal column joins the skull
 Craniosynostosis, when the bones in the skull fuse together
before the brain has stopped growing
 Dandy-Walker syndrome, when the fourth ventricle is
enlarged because of partial or complete closure of its
outlets
 Hydroanencephaly, a rare condition in which the brain's
cerebral hemispheres are absent and replaced by sacs filled
with cerebrospinal fluid
 Neural tube defects or spina bifida, when the spinal cord is
exposed at birth and is often lacking cerebrospinal fluid

13.  Schizencephaly, an extremely rare disorder characterized by abnormal
slits, or clefts, in the brain's cerebral hemispheres
 Vein of Galen malformations, abnormal connections between arteries
and the deep draining veins of the brain that develop before birth
 Syndromic forms - The most frequent are trisomies 13, 18, 9 and
tetrasomy 9p
 Intrauterine infection -rubella, cytomegalovirus, toxoplasmosis, and
syphilis = inflammation of the ependymal lining of the ventricular
system and the meninges in the subarachnoid space = obstruction of
CSF flow through the aqueduct or basal cisterns.
 Posthemorrhagic hydrocephalus occurs in approximately 35 percent of
preterm infants with intraventricular hemorrhage (IVH). It can be
obstructive, communicating, or both, and can be transient or
sustained, with slow or rapid progression.

14. SYMPTOMS
 Varied
 Infants are likely to have a
different disease
progression from that of
children and adults
 The most prominent sign
is a distended skull
 The sutures in infant
skulls are soft and not
fully developed, which
allows them to expand
upon increased pressure
from CSF accumulation.
 Nausea and vomiting
 Fussiness and irritability
 Poor appetite
 Sleepiness
 Seizures
 Developmental delay

15. ON EXAM
 Physical findings are due to the effects ICP.
 Brainstem distortions result in vital signs changes such as
bradycardia, systemic hypertension, and altered respiratory rate.
 The anterior fontanelle may become full or distended.
 Frontal bossing, an abnormal skull contour in which the
forehead becomes prominent.
 The scalp veins may appear dilated and prominent.
 Compression of the CN3 and 6 results in extraocular muscle
pareses leading to diplopia.
 Setting sun sign - Upward gaze due to pressure on the midbrain.
Appearance of the sclera visible above the iris.
 Fundoscopic examination may reveal papilledema.
 Spasticity of legs - stretching of the fibers from the motor cortex
around the dilated ventricles

16. INVESTIGATIONS
 Antenatal- fetal ultrasound as early as 14 weeks
 Infancy- head circumference crosses one or more grid
lines on the infant growth chart within a 4 week period
and there are progressive neuro signs.
 CT, MRI to detect ventriculomegaly
 Neuroimaging studies will also detect associated CNS
malformations or tumors.

17. TREATMENT
 1940s, before shunting was established, children with
hydrocephalus had a poor prognosis
 Most patients were not offered treatment, and only 20% of
children who did not undergo surgery for hydrocephalus reached
adulthood.
 Furthermore, children who survived had a 50% chance of having
permanent brain damage.
 Today, long-term treatments are surgical.
 Ventroperitoneal shunt placement in use since the 1950s, this
approach is considered the best treatment option in most cases
 Often requires long-term care and lifelong follow-up, especially
in children and neonates in whom there is a congenital cause.
 There is little use for medication in hydrocephalus.

19. PROGNOSIS
 The extent of the complications observed is dependent
upon the type of hydrocephalus, but patients with
epileptic seizures (approximately 30%) have the worst
clinical outcomes and, compared with patients who
did not develop seizures, are more likely to have an IQ
lower than 90.
 About 60% of children with hydrocephalus are able to
attend school (although many have difficulties), and
approximately 40% of children will lead relatively
normal lives

20. REFERENCES
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