Occipital encephalocoeles in 57 Nigerian children: a retrospective analysis

Fifty-eight patients with occipital encephalocoeles were retrospectively examined. These comprised about one-half of the cases seen and evaluated for treatment at the University College Hospital, Ibadan, Nigeria, between January 1973 and December 1987. There was a female-to-male preponderance of 2:1. Of the patients 91% were treated during infancy. Only one patient was precluded from surgery because his large ulcerated lesion was associated with severe microcephaly and neonatal sepsis, to which he succumbed. About four-fifths of the lesions exceeded 5 cm in diameter. The operative mortality was 6%, all deaths occurring in patients who were neonates at the time of surgery and whose hernia sacs contained brain substance. Clinically apparent hydrocephalus was more frequently encountered postoperatively, than preoperatively. Developmental delay was apparent in 5 of the 13 patients in whom developmental milestones were assessed during follow-up. For most patients, the follow-up period was short, possibly a reflection of the poor prognosis of the disease.