HYDROCEPHALUS

This is an increase in the volume of cerebrospinal fluid (CSF) within the ventricular system and may be communicating or non-communicating.

Clinical Features

  • There is a uniform enlargement of the head before birth causing obstructed labour or developing insidiously after birth.
  • There are prominent dilated scalp veins, and wide, bulging, and tense fontanelles.
  • Brow overhangs the roof of orbit
  • There is a cracked-pot sound when the head is percussed (McEwen’s sign)
  • A clear margin of sclera beneath the upper lid (setting sun sign), and wide sutures.
  • Nystagmus is common and transillumination is positive later.
  • In isolated hydrocephalus there is usually no neurological deficit. But if there was intrauterine infection then it may be accompanied by other defects.

Note: Some of these features develop over a period depending on the rate of increase in the head size.

Investigations

  • Skull x-ray is useful
  • Cranial ultrasound
  • CT scan where possible
  • Screen for congenital infections if necessary

Management

In order to prevent brain damage, early evaluation and diagnosis is essential. The baby therefore needs to be referred as soon as possible to a specialized unit.

Management – Operative (Specialized Neurosurgical)

  • A shunt from the ventricle to the peritoneal cavity is inserted in a specialized centre.
  • Contraindications for referral (Surgery)
    • Multiple congenital abnormalities.