hydrancephaly

Introduction

An extreme form of porencephaly.

Etiology

• may be caused by vascular infections or traumatic disorders after the 12th week of pregnancy

Epidemiology

• rare

Pathology

• cerebral hemispheres are absent & replaced by sacs filled CSF

Clinical manifestations

• infant may appear normal at birth
  • head size may be normal
  • spontaneous reflexes may all seem normal
• in a few weeks
  • the infant usually becomes irritable
  • muscle tone increased
• in a few months
  • seizures
  • hydrocephalus
• other signs/symptoms may include
  • visual impairment, growth retardation, deafness, blindness, spastic quadriparesis or paralysis, intellectual deficits
• diagnosis may be delayed for several months because early behavior appears to be relatively normal
• seizures
• myoclonus
• respiratory problems

Management

• no definitive treatment
• treatment is symptomatic & supportive
• hydrocephalus require ventriculoperitoneal shunt
• prognosis is generally poor
  • many die before age 1
  • in rare cases, children may survive for several years

More general terms

• porencephaly

Patient information

hydrancephaly patient information