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