HYDROCEPHALUS

Lambertus Josef F. Buga

 DEFINITION

• Derived from Greek word “Hydro” meaning “Water”, and

“Cephalus”, meaning “Head”.
• Hydrocephalus is excessive accumulation of cerebrospinal fluid
(CSF) resulting in abnormal widening of the spaces in the brain.
• Hydrocephalus is an abnormal accumulation of cerebrospinal
fluid within the ventricles of the brain
• Hydrocephalus is dilatation of the ventricular system.
 EPIDEMIOLOGY
• Estimated prevalence: 1–1.5%.
• Incidence of congenital hydrocephalus is ≈ 0.9–1.8/1000 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
 ETIOLOGY

Special form of
General Spesific Etiology
hydrocephalus
Non Normal pressure
Communicating Congenital hydrocephalus
Hydrocephalus (NPH)

Communicating Entrapped fourth

Acquired
Hydrocephalus ventricle
• Non Communicating Hydrocephalus
• hydrocephalus is due to blockage of the CSF circulation within the ventricular system.
• Such us Tumors in the region of the foramen of Monro
• Communicating Hydrocephalus
• hydrocephalus is due to impaired circulation of the CSF after it leaves the ventricular system,
• such as from fibrosis and scarring of the basal cisterns because of meningitis, or impaired function of the arachnoid granulations from scarring due to previous
subarachnoid hemorrhage.
• Congenital Hydrocephalus
• Present at birth and may be caused by environmental influences during fetal development or by genetic factors.
• Intra uterine infections : Toxoplasmosis, Rubella, Cytomegalovirus
• Congenital Malformations : Arnold Chiari malformations, Agenesis of foramina Monro, Arteriovenous malformation, Brainstem malformation, Cranial defects, Dandy
walker malformations
• Acquired Hydrocephalus
• Develops at the time of birth or at some point afterward.
• This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.
• Infections : Meningitis,TORCH,Cysticercosis, Brain abscess;
• Post haemorrhagic : Head injury, Subarachnoid haemorrhage 
• Mass lesions : Posterior fossa tumors, Astrocytoma, Ependymoma, Sagittal sinus thrombosis, Hypervitaminosis A
• Idiopathic normal pressure hydrocephalus (NPH)
• a form of communicating hydrocephalus with ventricular enlargement but normal OP on LP that occurs spontaneously, without any identifiable antecedent event that
may have caused meningeal scarring.
• It accounts for about one-third of the cases of adult-onset communicating hydrocephalus
ANATOMY OF VENTRICULAR SYSTEM
CIRCULATION OF CSF

 The choroid plexuses in the

ventricles produce most of the
cerebrospinal fluid (CSF)
 The circulating fluid volume (150 ml
[5 fl oz]) is exchanged constantly,
with a daily CSF production volume
of approx 500 ml [17 fl oz]
 The CSF serves as a cushion to
protect the CNS from mechanical
forces, and reduces the weight of the
CNS (by creating a buoyancy which
causes a 97 % weight reduction from
1,400 g to 45 g [49 oz to 1 6
oz]),Supports the metabolism of the
CNS, removes toxic substances,
transports hormones (e g leptin), and
facilitates electrical conductance
 PATHOGENESIS
• Obstruction of CSF flow
• Tumors may form in the region of the foramen of Monro, most commonly a colloid cyst of the third ventricle, and obstruct CSF flow, causing hydrocephalus and positional headache.
• Stenosis of the cerebral aqueduct may cause dilatation of the third and lateral ventricles with a normalsized fourth ventricle.
• Obstructive hydrocephalus may develop when the outlet foramina of the fourth ventricle are occluded. When the outlet foramina fail to develop normally, the fourth ventricle becomes
massively dilated, creating a cystic structure in the posterior fossa (Dandy-Walker syndrome). Very rarely, hydrocephalus is caused by overproduction of CSF, such as by a papilloma of
the choroid plexus.

• Impaired resorption
• The impaired CSF circulation or decreased absorption transmits increased back pressure into the system, causing compensatory ventricular dilatation.
• Hydrocephalus ex vacuo
• a compensatory mechanism for loss of brain volume
• Indicated enlargement of ventricles secondary to parenchymal damage –
• Associated with aging, head trauma, severe infection, hypoxia or ischemic insults.
• Also following chemotherapy and radiotherapy has been noted
• Structural abnormalities of brain like calpocephely, holoprocencephaly and agenesis of corpus callosum are also associated.
SYMPTOMS Newborn
• OFC increasing rapidly more than 97th centile
• Headache and vomiting – more in • Bulging AF with association of scalp signs
morning, better on sitting and it becomes • Poor feeding
continuous as hydrocephalus progresses • Frequent vomiting
• Urinary incontinence presenting as • Reduced activity
urgency, frequency which follows • Association spina bifida with or without
diminished awarness to urinate as it meningocele or meningomyelocele
progresses Infant
• Blurred vision due to papilloedema • Large head size
• Drowsiness • Poor feeding

• Horizontal diplopia – 6th nerve palsy • Irritability

• Decreased acitivity
• Spasticity in lower limbs – difficulty in
walking • Lack of interest in surroundings
• Vision problem
Toddler & children
• Headache and vomiting
• Neck pain (cerebellar tonsil herniation)
• Horizontal diplopia (6th nerve)
• Lethargy and drowsiness
• Urinary incontinence
• Memory loss
• Progressive macrocephaly
• Gait disturbances
  Children (with rigid cranial vault) and adults

SIGNS 

Papilledema
Gait changes
 Upgaze and/or abducens palsy.
 NPH classically causes a triad of dementia, gait disturbance, and urinary incontinence
 Abnormalities in head circumference/ Macrocephaly (OFC=Occipito Frontal Circumference)  increase in occipito
frontal circumference more than 2cm/month in first 6months
 Fontanelle full and bulging
 Frontal bossing (protuberance of the frontal bone manifesting as a prominent forehead)
 Enlargement and engorgement of scalp veins: due to reversal of flow from intracerebral sinuses, due to increased
intracranial pressure
 Skin over scalp is thin and shiny
 6th nerve (abducens) palsy: the long intracranial course is postulated to render this nerve very sensitive to pressure
 Hyperactive reflexes
 Irregular respirations with apneic spells
 Pyramidal signs in lower limbs - Pyramidal fibres are stretched giving plantar extensor reflex and exaggerated DTR
s with increased tone
• “sunset sign” (upward gaze palsy)
• Macewen’s sign: cracked pot sound
on percussing over-dilated
ventricles
• Transillumination - > 2-2.5cm in
frontal and over 2cm in occipital in
abnormal
 High pitch cry
 Splaying of cranial sutures (may be
seen on plain skull X-ray)
• Bradycardia
• Hypertension
 INVESTIGATION
FINDING ETIOLOGY CONFIRMATING OF DIAGNOSIS
• TORCH screening • Xray  Enlargement of skull,
• CSF analysis for protein levels and Widening of sutures
opening pressure • Silver bitten appearance
• Ballooning of sella turcica
• Calcification in toxoplasmosis and
cytomegalo
• CT Scan
• MRI
• Silver beaten appearance : These
are rounded zones of bony
Attentuation of the cranial bones
Caused by pressure from the
cerebral cortical gyri, resulting
from premature closure of cranial
sutures in infants and by
extension, increased intracranial
pressure.
There can be unilateral dilation of ventricle Ballooning of frontal and third
in Obstruction of foramen of Monroe ventricle, Mickey mouse ventricles
MANAGEMENT
 MANAGEMENT

MEDICAL TREATMENT SURGICAL

Acetazolamide : 25 mg/kg/day PO Endoscopic third ventriculostomy

until 100 mg/kg/day
Furosemide: 1 mg/kg/day
Shunt
• ventriculoperitoneal (VP) shunt
• ventriculo-atrial (VA) shunt
(“vascular shunt”)
• Torkildsen shunt
• lumboperitoneal (LP) shunt
Endoscopic third ventriculostomy
COMPLICATIONS OF VARIOUS SHUNTS

• Obstruction
• Disconnection at a junction, or break at any point
• Infection
• Hardware erosion through skin
• Seizures
• Extraneural metastases of certain tumor
• Allergy
 PROGNOSIS
• The extent of the complications observed is dependent upon the type of
hydrocephalus
• 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),
• Approximately 40% of children will lead relatively normal lives
 REFERENCE
• Campbell William W., Barohn Richard J. Hydrocephalus in DeJong’s
The Neurologic Examination. 8th Edition. 2020. Chap.50:p1435-36
• S. Hombach-Klonisch, T. Klonisch, J. Peeler. Ventricular System In
Sobotta : Clinical Atlas of Anatomy. 1 st Edition. 2019. Chap.12:p572-73
• Winn Richard H. Hydrocephalus in Youmans & Winn Neurological
Surgery. 7th Edition. Vol.2, Sec.VII:Part 4:p6028-34