spinal dysraphism and hydrocephalus

Spinal Dysraphism (Spin Bifida)

spina bifida occulta
congenital absence of a spinous process & a variable amount of lamina without visible exposure of meninges or neural tissue
-20-30% of north Americans
-often an incidental finding
-usually of no clinical importance when it occurs alone
-may be associated with diastematomyelia, teathered cord, lipoma, dermoid tumor
-the defect may be palpable & there may be cutaneous manifestations

spina bifida aperta(cystica)

meningocele:-
congenital defect in vertebral arches with cystic distension of meninges, but no abnormality of neural tissue
myelomeningocele:-
congenital defect in vertebral arches with cystic distension of meninges &structural or functional abnormality of spinal cord or cauda equina

-0.1-0.2%

-2-3% with previous birth of MM
-6-8% after 2 affected children
-65-85% of MM associated with hydrocephalus
-folic acid difficiency
-prenatal dignosiselevated maternal serum AFP between 15-20 weeks of gestation + ultrasound

Management
-size of the defect
-ruptured or unruptured
-neurological assessment
-assessment of the other systems


Hydrocephalus
Dilatation of the ventricles due to excessive production, obstruction in the pathway, or defective absorption of CSF

1-obstructive (non-communicating)-the block is proximal to the arachnoid granulations e.g cerbellar tumors, congenital aqueduct stenosis
2-non-obstructive (communicating)-the block at the level of arachnoid granulations e.g meningitis

Signs & symptoms

In young children
1-cranial enlargement at a rate greater than facial growth
2-irritability, poor head control, nausea & vomiting
3-enalargement & engorgement of scalp veins
4-fontanelle full & bulging
5-Macewens sign---cracked pot sound on percussing over dilated ventricles
6-6th nerve palsy
7-setting sun sign---upward gaze palsy---Parinauds syndrome from pressure on region of suprapineal recess
8-hyperactive reflexes
9-irregular respiration with apneic spells
10-splaying of cranial sutures

In older children & adults

Symptoms of increased intracranial pressure


Investigations
1-skull x-ray
2-U\S
3-CT scan
4-MRI

Treatment

Always surgical

1-diuretics

-acetazolmide 25 mg/kg/day
-furosemide 1mg/kg/day
2-aspiration through the anterior fontanel- therapeutic & diagnostic
3-diversion procedures-shunting
-ventriculo-peritoneal
-ventriculo-pleural
-ventriculo-atrial
-lumbo-peritoneal
4-endoscopy

Best Wishes;

Saif AlDeen Adil Kamil










Lec.4 Neurosurgery Dr.Bassam

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