Neural Tube Defect
It occurs when the neural tube doesn’t close
properly
Neural tube form the early brain and spinal
cord with it’s coverage
It occurs early in pregnency and
is the most dangerous type of
congenital anomalies
Types
Hydrocephalus
Spina bifida
Anencephaly or microcephaly
Encephalocele
Hydrocephalus
DEFINITION
• Hydrocephalus is an excessive CSF in the
ventricular system either due to obstruction in
the pathway or rarely due to excessive
formation
CSF FORMATION AND ABSORBTION
CSF forms at rate 500ml/day
The function of CSF is protection and
neutrition of CNS cells
TYPES OF HYDROCEPHALUS
1) Obstructive hydrocephalus: is obstruction of
the CSF within the ventricular system
2) Communicating hydrocephalus: Obestruction
outside the ventricular system i.e. ventricular
CSF communicating with subaracnoid space
3) Normal pressure hydrocephalus or Hakim-
Adam syndrome
4) Multi-locular hydrocephalus
CAUSES OF HYDROCEPHALUS
1. Obstructive:
Congenital:
Aqueductal stenosis
Dandy-Walker syndrome
Arnold-Chiarri malformation
Vein of Galen malformation
Acquired:
Aqueductal stenosis
Intraventricular hemorrage
Colloid cyst
Pineal body tumor
Posterior fossa tumor
2. Communicating:
Thickening or Leptomeninges due to:
Infection
Trauma
Postoperative
Carcinomatous meningitis
Increase CSF viscosity e.g. high protein content
Excessive CSF production due to Choroid plexus papilloma (rare)
CLINICAL FEATURES
1. Infant and young children (before closure of anterior
fontanelle):
Delayed milestones
Increase head circumference
Wide bulge or tense anterior fontanelle
Thin scalp
Dilated scalp veins
Craniofacial dispropotion
Sunset appearance of the eye due to tectum comprsssion
by 3rd ventricle
2. Adult hydrocephalus (after closure of anterior fontanelle)
Acute onset (manifestation of increase intracranial tension):
Headache
Vomiting
Blurring of vision due to papilledema
Seizures
Deterioration of conscious level
Gradual onset:
Dementia
Gaint disturbance
Urine incontinence
INVESTIGATIONS
Skull X-ray:
Skull size and suture width
CT scan:
The best investigation give the pattern of ventricular enlargement
and helps to determine the type either obstructive or
communicating and the cause of obstruction
Trans-fontanelle U/S:
If CT isn’t availabe
TREATMENT
1. Shunt operation
2. 3rd Ventriculostomy
The shunt operation is shunting CSF to other
part in the body by shunt device
Shunt device consist of:
1. Ventricular catheter
2. Reservoir
3. Distal catheter
According to site of the distal
catheter insertion gives the type of
the shunt:
1. Ventriculo-peritoneal shunt
2. Ventriculo-atrial shunt
3. Ventriculo-pleural shunt
COMPLICATIONS OF SHUNTING
1. Infection
2. Obstruction
3. Overdrainage
PROGNOSIS
• Depend on brain mental state and early
intervention
MCQs
1. Definition of hydrocephalus:
a. Excessive CSF in ventricular system
b. Excessive fluid in the ventricular system
c. Excessive CSF in subarachnoid space
d. Excessive CSF in subdural space
2. In the brain there is:
e. Three ventricles
f. Two ventricles
g. Five ventricles
h. Four ventricles
3. Aqueduct of sylvius present between:
a. Lateral and 3rd ventricle
b. 3rd and 4th ventricle
c. Two lateral ventricle
d. 4th ventricle and subarachnoid space
4. The most common cause of obstructive hydrocephalus is:
e. Aqueductal obstruction
f. Foramen of monro obstruction
g. Subarachnoid hemorrage
h. Increase CSF viscosity
5. Clinical feature of hydrocephalus in infant:
i. Headache
j. Vomiting
k. Blurring of vision
l. Wide bulge anterior fontanell
6. Sunset appearance is due to:
a. Posterior fossa compression
b. Cortical compression
c. Brain tectum compression
d. Tentorial herniation
7. The best investigation for hydrocephalus:
a. CT head
b. Skull x-ray
c. Ulterasonography
d. MRI
8. Ventriculoperitoneal shunt:
a. Is shunt between lateral ventricle and pleural cavity
b. Is shunt between lateral ventricle and right atrium
c. Is shunt between lateral ventricle and peritoneal
cavity
d. Is shunt between lateral ventricle and ureter
9. The ventricular catheter inserted in:
a. Right lateral ventricle
b. 3rd ventricle
c. 4th ventricle
d. Subarachnoid space
Thank You