Hydranencephaly in Malawian children

Abstract

Objective: To show that not all big heads in children are due to hydrocephalus.
Setting: The Queen Elizabeth Central Hospital (QECH), Blantyre, the teaching hospital of Malawi College of Medicine. It is the biggest and leading hospital in Malawi and the major referral centre for the whole country.
Design: A prospective study was made of all children who presented at QECH, Blantyre with the diagnosis of hydrocephalus during the study period.
Subjects: Fifteen cases of hydranencephaly were encountered.
Intervention: All cases of abnormally large heads were investigated with cranial ultrasonography, a few children had air ventriculography and CT scan of the brain. All patients had ventricular aspiration of cerebral spinal fluid (CSF) to verify the colour and condition of the fluid before performing ventriculo-peritoneal shunting for which our locally
made Malawi unishunt was used.

Results: In hydranencephaly, the brain was found replaced by large fluid-filled cavity in contrast to hydrocephalus where brain was present and ventricular dilatation was encountered. Ventriculo-peritoneal shunting benefited children with hydrocephalus but not those with hydranencephaly.
Conclusion: Hydranencephaly is one of the causes of abnormal head enlargement in children often confused with hydrocephalus. It is important to recognise hydranencephaly to avoid unnecessary operation which does not help the patient.