HYDROCEPHALOUS

By Dr. Sudarshan Reddy . K

Department of Paediatrics
 Normal flow of CSF

FORMATION :
Bulk of csf is formed from choroid plexus
of lateral ventricles and lesser amount by
choroid plexus of third and fourth ventricle.
How CSF Flows
CSF from lateral ventricle

Monro

Third ventricle

Cerebral aqueduct

Luschka
Fourth ventricle
Magendie
 From 4 th ventricle the csf passes to the sub
arachnoid space through the median and lateral
appertures of fourth ventricle.
 csf is absorbed through arachnoid villi and
granulation thus drained into cranial venous
sinuses.
 Other sites of absorption : perineural lymphatics
of 1, 2 and 8 th cranial nerve and also veins
related to spinal nerves
Control of csf secretion
 Stimulation of the adrenergic system diminishes
CSF production.
 whereas excitation of the cholinergic nerves may
double the normal CSF production rate.
 In a normal child, approximately 20 mL/hr of
CSF is produced. The total volume of CSF
approximates 50 mL in an infant and 150 mL in
an adult.
 DEFINATION
Hydrocephalus is not a specific disease; it
represents a diverse group of conditions that
result from impaired circulation and/or
absorption of CSF or, in rare circumstances, from
increased production of CSF by a choroid plexus
papilloma
 TYPES
-OBSTRUCTIVE / NONCOMMUNICATING
-NON OBSTRUCTIVE /COMMUNICATING
 Hydrocephalus resulting from obstruction within
the ventricular system is called obstructive or
noncommunicating hydrocephalus.
 Hydrocephalus resulting from obliteration of the
subarachnoid cisterns or malfunction of the
arachnoid villi is called nonobstructive
or communicating hydrocephalus.
 ETIOLOGY
COMMUNICATING
 Choroid plexus papilloma
 Meningeal malignancy
 Meningitis
 Posthemorrhagic
 Achondroplasia
 Benign enlargement of subarachnoid space
 NONCOMMUNICATING
 Aqueductal stenosis
 Infectious*
 X-linked
 Mitochondrial
 Autosomal recessive
 Autosomal dominant
 L1CAM mutations
 Chiari malformation
 Dandy-Walker malformation
 Klippel-Feil syndrome
 Mass lesions
 Abscess
 Hematoma
 Tumors and neurocutaneous disorders
 Vein of Galen malformation
 Walker-Warburg syndrome
 Arnold chiari malformation
Chiari malformation consists of 2 major
subgroups :

Type I : The deformity consists of

displacement of the cerebellar tonsils into
the cervical canal, Syrinx of the spinal cord,
especially the cervical region should be looked
for on MRI imaging.
Type II : Chiari malformation is characterized by
progressive hydrocephalus with a
myelomeningocele. This lesion represents an
anomaly of the hindbrain, probably owing to a
failure of pontine flexure development during
embryogenesis, and results in elongation of the
fourth ventricle and kinking of the brainstem,
with displacement of the inferior vermis,
pons, and medulla into the cervical canal.
Sagittal MR scan of a patient
with Chiari malformation
type I. Cerebellar tonsils are
displaced through the foramen
magnum (white bar) to the lower
aspect of C2 with clear crowding at
the foramen. A syrinx (white
asterisk) is visible extending from
C3 to T2. (FromYassari R, Frim D:
Evaluation and management of the
Chiari malformation type 1
 Dandy walker malformation
 The Dandy-Walker malformation consists of a cystic
expansion of the fourth ventricle in the posterior
fossa and midline cerebellar hypoplasia, which
results from a developmental failure of the roof of
the fourth ventricle during embryogenesis.
 Approximately 90% of patients have hydrocephalus,
and a significant number of children have associated
anomalies, including agenesis of the posterior
cerebellar vermis and corpus callosum.
 CLINICAL FEATURES
In an infant :
 An accelerated rate of enlargementof the head is
the most prominent sign.
 The Anterior fontanel is wide open and bulging
 The scalp veins are dilated.
 Setting-sun eye sign
 CLINICAL FEATURES
 In an older child, the cranial sutures are less
accommodating so that the signs of hydrocephalus
may be subtler.
 Irritability, lethargy, poor appetite, and
vomiting are common to both age groups,
and headache is a prominent symptom in older
patients.
 Serial measurements of the head circumference
often indicate an increased velocity of growth.
 CLINICAL FEATURES
 Percussion of the skull might produce a cracked pot
sound or Macewen sign, indicating separation of the
sutures.
 A foreshortened occiput suggests Chiari
malformation, and a prominent occiput suggests the
Dandy-Walker malformation.
 Papilledema, abducens nerve palsies, and pyramidal
tract signs, which are most evident in the lower
extremities, are apparent in many cases.
 Investigation
HISTORY :
 Familial cases suggest X-linked or autosomal
hydrocephalus secondary to aqueductal stenosis.
 A past history of prematurity with intracranial
hemorrhage, meningitis, or mumps encephalitis is
important to ascertain.
 Multiple café-au-lait spots and other clinical features of
neurofibromatosis point to aqueductal stenosis as the
cause of hydrocephalus
 Investigation
Examination :
 skull and spine.
 The occipitofrontal head circumference is recorded
and compared with previous measurements.
 The size and configuration of the anterior fontanel
are noted, and the back is inspected for abnormal
midline skin lesions, including tufts of hair, lipoma,
or angioma that might suggest spinal dysraphism.
 Investigation
 Inspection of the eyegrounds is mandatory
because the finding of chorioretinitis suggests
an intrauterine infection, such as toxoplasmosis,
as a cause of the hydrocephalus.
 Papilledema is observed in older children but
is rarely present in infants because the cranial
sutures separate as a result of the increased
pressure.
Plain skull films :
 Separation of the sutures,
 Erosion of the posterior clinoids in an older
child.
 Increase in convolutional markings (beaten-
silver appearance) on the inside of the skull with
long-standing increased ICP.
Silver beaten type of skull
 The CT scan and/or MRI along with
ultrasonography in an infant are the most
important studies to identify the specific cause
and severity of hydrocephalus.
 TREATMENT
MEDICAL TREATMENT :
 Acetazolamide
 Furosemide
 Mannitol
 3 percent normal saline

 Medical treatment can provide temporary relief by

reducing the rate of CSF production
 Treatment
Surgical treatment :
- ventriculoperitoneal shunt.
- ventriculo atrial shunt
- ventriculo pleural shunt
- Endoscopic third ventriculostomy
The major complications of shunting :
1. occlusion
2. Bacterial infection
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YOU YOU

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