Clinical features of infantile hydrocephalus

Clinical features of paediatric hydrocephalus include:

• characteristic macrocephaly - enlargement before birth may obstruct labour. Usually, it is slowly progressive and the head may attain a huge size with a circumference of 30 inches or more. Enlargement occurs in all diameters. The cranial sutures separate to accommodate the expanding ventricles so that signs of raised intracranial pressure are not an immediate feature.
  • the anterior fontanelle is much enlarged and tense
  • in extreme cases, the head may be translucent and yield a fluid thrill on percussion
  • scalp veins are prominent
• irritability
• poor feeding
• failure to thrive
• developmental delay
• convulsions and mental impairment are common
• the lids are retracted, the eyes are depressed forwards and downwards, and upward gaze is impaired - the setting sun appearance.
• there is often optic atrophy, under-development of the limbs, and spastic diplegia of varying severity
• apnoeic spells (1)

Reference:

• (1) Kandasamy J, Jenkinson MD, Mallucci CL. Contemporary management and recent advances in paediatric hydrocephalus. BMJ. 2011;343:d4191