Giant Encephalocele in Sokoto, Nigeria: A 5-Year Review of Operated Cases

Abstract

Objective: Encephalocele is a common congenital malformation of the central nervous system; however, giant encephaloceles are rare. The use of folic acid supplementation and termination of pregnancies, which are prenatally diagnosed with encephaloceles and other congenital malformation of the central nervous system, has significantly reduced the occurrence of this type of congenital malformation, especially in developed countries.

Methods: This was a retrospective review over a 5-year period from January 2006 to December 2010 at the Department of Neurosurgery, Regional Centre for Neurosurgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. The records of patients with giant encephalocele were retrieved from the case notes of patients who had excision and repair for encephalocele.

Results: Seventy-three patients had excision and repair of encephalocele over the study period. However, the records of only 50 patients were retrieved. Fourteen (28%) of the 50 whose records were retrieved had giant encephalocele. There were 4 male and 10 female patients (1:2.5). Thirteen (92.9%) had the lesion located in the occipital region, whereas in 1 patient (7.1%) the lesion was at the vertex. Three (21%) of the cases had microcephaly, 1 (7.1%) had macrocephaly, and 1 (7.1%) developed postoperative hydrocephalus. The average size of defect was 2.43 cm, and the size of the lesion ranged from 12 × 6 cm to 40 × 50 cm. The average maternal age was 20.3 years (n = 6), and the paternal age was 29 years (n = 4). Four out of 7 (57%) mothers had febrile illness in early pregnancy. Seven out of 10 patients (70%) did not have antenatal care.

Conclusions: This condition is more common in children conceived during the period when farm products are yet to be harvested, and whose mothers did not attend antenatal care visits. It is recommended that mothers should be educated on the necessity of preconception folic acid. Implementation of a national strategy on food fortification is also advised. We recommend surgery after the first month of life to reduce poor early postoperative outcomes. In developing countries and centers with suboptimal pediatric intensive care units, surgical intervention is preferred after the first month of life with good temperature control, adequate fluid replacement following rupture of the sac, blood transfusion availability, and, only if necessary, complex cranial reconstruction.

Keywords: Congenital malformation; Encephalocele; Giant; Nigeria; Sokoto.