excessive accumulation of CSF associated with progressive ventricular dilatation pathophysiology/etiology increased produc...
- excessive accumulation of CSF associated with progressive ventricular dilatation
- pathophysiology/etiology
- increased production or CSF e.g. choroid plexus papilloma
- decreased absorption of CSF e.g. hyperplasia of arachnoid villi, infection/hemorrhage destroying arachnoid villi
- obstruction to flow of CSF e.g. congenital malformations (Dandy-Walker, Arnold-Chiari), masses, infections, congenital bone defects
Clinical Signs
- in utero - large head
- ventricular distention leads to stretching of the pathways surrounding ventricles which may cause
- ataxia,
- spasticity (lateral ventricle),
- hypothalamic dysfunction (3rd)
- impaired vertical gaze (4th)
Acute (increased ICP)
- irritability, lethargy, loss of appetite, vomiting
- large fontanelle; splayed sutures
- headache
- cranial nerve deficits
- herniation/coma
Chronic
- onset < 2 years: macrocephaly and excessive rate of head growth
- ataxia, spasticity
- papilledema, optic atrophy, impaired upward gaze,
- endocrine dysfunction (primarily causing growth failure)
Diagnosis
- prenatal ultrasound
- post natal ultrasound/CT/MRI
Treatment
- medical – treat underlying cause; acetazolamide (transiently decreases CSF production)
- surgical – remove lesion; ventriculoperitoneal shunt
