HydrocephalusAmaan Mohiuddin

Gary Oh

INTRODUCTIONHydrocephalus 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

EPIDEMIOLOGYIncidence of congenital hydrocephalus 2-

3/1000 live birthsIncidence equal in males and females15-25% 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

PATHOPHYSIOLOGYCSF 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

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

CAUSES, DIFFERENTIAL DXInherited

MyelodysplasiaAtresia of formen of

MonroNT defectEncephaloceleSpina bifidaGenetic abnormalitiesAqueductal stenosisDandy-Walker syndromeOther cerebral and

spinal malformations

Acquired Tumor Meningitis Infection Hemorrhage Arachnoid Cyst Posterior Fossa

Cyst TBI Idiopathic

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

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.

SYMPTOMSVaried 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 vomitingFussiness and

irritabilityPoor appetite SleepinessSeizuresDevelopmental delay

ON EXAMPhysical 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

INVESTIGATIONSAntenatal- fetal ultrasound as early as 14

weeksInfancy- 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 ventriculomegalyNeuroimaging studies will also detect

associated CNS malformations or tumors.

TREATMENT1940s, 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.  

PROGNOSISThe 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

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