Neurodevelopmental Regression

Hydrocephalus is pathological accumulation of CSF resulting from impaired flow, impaired absorption or, rarely, excess production. It causes progressive ventricular enlargement and may raise intracranial pressure.

Causes include congenital aqueductal obstruction, neural tube defects, postinfectious disease, intraventricular haemorrhage and intracranial masses.

In infants:

• Rapidly increasing head circumference
• Bulging tense fontanelle
• Splayed cranial sutures
• Sunset eyes
• Irritability
• Vomiting
• Poor feeding

Older children present with:

• Headache
• Morning vomiting
• Papilloedema
• Diplopia
• Gait disturbance
• Declining school performance
• Reduced consciousness

• Serial head circumference is highly valuable in infants.

• Cranial ultrasound can assess ventricular size while the fontanelle remains open.

• MRI provides the best anatomical assessment and identifies the site and cause of obstruction.

• CT is useful where rapid imaging is required but exposes the child to ionizing radiation.

• Symptomatic hydrocephalus requires neurosurgical management.

• Definitive options include ventriculoperitoneal shunting and endoscopic third ventriculostomy, sometimes combined with choroid plexus cauterization in selected infants.

• Acute deterioration with reduced consciousness, bradycardia, hypertension or abnormal pupils requires emergency management of raised intracranial pressure and immediate neurosurgical review.

• A child with a shunt presenting with headache, vomiting, reduced consciousness or fever should be assumed to have shunt obstruction or infection until excluded.