HYDROCEPHALUS

BY LEX-MEDLIFE CENTER
(DR I. MWANGALA + DR M. KAZEVU
 OBJECTIVES
 DEFINITION
 CSF CIRCULATION
 CAUSES
 CLASSIFICATION
 PATHOPHYSIOLOGY
 CLINICAL FEATURES
 DIFFERENTIAL DIAGNOSIS
 INVESTIGATION
 TREATMENT
 COMPLICATIONS
 DEFINITION
 Is a disorder characterized by dilatation of ventricles due to blockage
of cerebrospinal fluid flow (CSF) or due to increased secretion or due
to defective absorption of CSF
 FUNCTIONS OF CSF
 Provide a cushioning effect on the CNS and dampens the effects of
trauma.
 Removal of metabolites from the CNS.
 Provides a stable ionic environment for the CNS under normal
conditions.
 Brain and spinal cord float in CSF whose buoyant effect results in
reduction of traction exerted upon the nerves and blood vessels
connected to these CNS structures.
 CAUSES
I. An excess of CSF production - (in rare condition of choroid plexus
papilloma and choroid plexus Ca, Choriod plexus congestion as in
meningitis.
II. CSF flow obstruction (an obstructive hydrocephalus) - aqueductal
stenosis, brain lesion and tumour, infections (meningitis), intraventricular
bleeds, Dandy Walker cyst
III. Failure of reabsorption (a communicating hydrocephalus) -
obstruction at the arachnoid granulations, sinus thrombosis, arachnoiditis,
Congenital infection especially toxoplasmosis, Leukemic infilteration,
Subarachnoid space adhesions (Rare) due to: Post hemorrhagic or Post
meningitic
IV. Associated abnormalities- Dandy-Walker syndrome, Arnold-Chiari
malformation, Aqueductal stenosis
 CLASSIFICATION
Classification I of Hydrocephalus
Communicating type: Ventricles communicate freely into the
subarachnoid space.
 Here there is defective absorption of CSF following any inflammation, subarachnoid
haemorrhage or trauma.
Normal pressure hydrocephalus- Occur in cerebral atrophy, ventricles
are dilated but the pressure is not raised.
 Aka Hydrocephalus ex vacuo,Cause of NPH may be known but most often it’s
idiopathic.
Noncommunicating type: Obstruction is in the ventricle or its exit, due to
any tumors or any inflammatory process.
 Movement of CSF out of the ventricular system is impeded (e.g. blockage at cerebral
aquaduct/foramina of 4th ventricle- foramina of luschka and magedie)
 CLASSIFICATION II OF HYDROCEPHALUS

Congenital Hydrocephalus - Presents at birth caused by complex

interaction of genetic and environmental factors during fetal
development
 Causes: Intrauterine infections/conditions, Congenital malformations,
Intracranial hemorrhage
Acquired hydrocephalus – develops after birth due to neurological
conditions e.g. head trauma, Infection, Post-intraventricular
hemorrhage, Brain tumors
 PATHOPHYSIOLOGY
 As a results of an imbalance between production, circulation and
absorption of CSF, CSF continues to accumulate leading to
progressive dilatation of the subarachnoid channels,
 Increase in CSF pressure is transmitted to the ventricular system
resulting in ventriculomegaly.
 Ependymal lining of ventricles is disrupted resulting in periventricular
ooze and hence periventricular compression of adjacent white matter.
 Ventricular enlargement causes displacement of primary cerebral
arteries and a reduction in the calibre and number of secondary and
tertiary vessels causing diminished blood flow and ischemia.
 CLINICAL FEATURES
Depends on age of pt, causative factor, associated malformations or
cerebral insult and severity and progression of the disease
Newborn and Infant
Large head with progressive increase in size (increasing head circumference
on serial measurement).
Fontanels are widely opened & bulging, Sutures are widely separated.
Dilated scalp veins.
Eyes deviated downwards ~ sunset appearance
Skull percussion~ cracked pot sound (Macewen sign)
 Poor feeding and failure to thrive
Lethargy, somnolence and irritability.
Delayed milestones and difficulty in head control
 Older Child
Older children have a more acute presentation due to inability of fused
cranium to expand
Presents with a triad of: severe headache, vomiting and lethargy.
Papilloedema on fundoscopy due to ↑ICP
Skull contour becomes abnormal and forehead is prominent
Delayed motor and cognitive development and subtle behavioural changes
are noted.
 A mild spastic diplegia (pyramidal tract signs) with positive Babinski sign
can also be elicited.
Features of raised ICP mainly in adult, lethargy, irritability, ataxia,
papilloedema, blindness, ophthalmoplegia, abducens palsy and respiratory
failure
 DIFFERENTIAL DIAGNOSIS
 Familial macrocrania
 Subdural fluid- Haematoma and Effusion
 Megalocephaly
 Gigantism
 Benign enlargement of subarachnoid space
 Soto’s syndrome (Large body, Large brain, Mental retardation)
 INVESTIGATION
 Skull x-ray - May show separation of sutures
Brain ultrasound - Done when fontanelles are open and sutures are sprung
out
o May show dilated ventricles
o May show cause like Intraventricular hemorrhage especially in prematurity
o May show 4th ventricle tumor
CT or MRI - Investigations of choice in older children and adults as there is
no fontanelle open for USS. It will show degree of hydrocephalus and the
cause
Carotid angiography – sylvian points pushed out >3-6mm
Other Diagnostic Studies
o CSF evaluation: protein and cell content is important prior to shunt placement in
postmeningitic hydrocephalus. Fat laden cells are indicative of brain damage in post-
infection states
o Cerebral blood flow and Doppler studies:
o Fundoscopy – Exclude papilloedema in ↑ICP
 TREATMENT
Establish the precise etiological diagnosis, malformation, clinical
course and severity
MEDICAL: DIURETICS
 Acetazolamide- Reduces active secretion of CSF by choroid plexus, but not
from brain parenchyma or ependymal cells ling the ventricles
 Furosemide- May reduce intracranial water content
 Diuretics may temporarily slow down growth of hydrocephalus
 Osmotic diuretics (mannitol, urea and glycerol)
 Increase Cerebrospinal Fluid Absorption (Hyaluronidase, urokinase,
streptokinase)
 Removal of Cerebrospinal Fluid
SURGERY
 Tapping of the lateral ventricles.
 Ventriculocysternostomy using polythene catheters— Torkildsen
operation
 Ventriculo-peritonel shunt
 Ventriculo-pleural shunt
 Ventriculo-atrial shunt (Vascular shunt)
 3rd ventriculostomy – From the 3rd ventricle to the SAS
 Ventriculo-gall bladder shunt
 Torchedsen shunt
 COMPLICATIONS
Acute
 Infection,
 Slit ventricular syndrome (small ventricles formed in shunt).
Long term
 Shunt becoming short as the child is growing,
 Blocked shunt,
 Dislodged shunt and
 Shunt nephritis
THANK YOU