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.


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


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.