Background: Encephalocoeles are herniation of the brain and its meninges via a midline cranial vault defect or at the base of the skull, at the site of local mesenchymal disruption. It accounts for 10% to 20% of all the cranial and spinal dysraphisms found in 1/5000 live births.Method: It is a 5 year retrospective study on 42 cases of encephalocoele managed in a Nigerian Tertiary Medical Centre. We extracted the clinical findings and surgical management outcomes from the patient's case notes.Result: Ages at presentation were Neonates (24, 57.1%), ≤3 months (35, 83.3%), with mode of 2 days. Males (12, 28.6%), females (30, 71.4%), with M:F=1:2.5. Posterior (occipital) lesions (36, 85.7%), anterior (6, 14.3%). Variable size lesions (33, 78.6%), giant encephalocoeles (9, 21.4%). Hydrocephalus (16, 38.1%) included pre-excision HCP (12, 75% of 16) and post excision HCP (4, 25% of 16). Available neuroimaging was TFUSS (all, 100%), CT Scan (20, 47.6%), MRI (2, 4.8%). Surgery of excision and repair (36, 85.7%), combined craniofacial repair (6, 14.3%), additional ventriculoperitoneal shunt (16, 38.1%). Post-operative complications included CSF leak (2, 4.8%), superficial surgical site infection (3, 7.1%), meningitis (1, 2.4%), shunt infection (4, 9.5%), shunt obstruction (7, 16.7%). Outcomes were good (38, 90.5%), blind (3, 7.1%), vegetative (1, 2.4%), death (1, 2.4%).Conclusions: Encephalocoeles are an uncommon congenital type of neural tube defect containing herniated brain tissue. Posterior (occipital) lesions are commoner (and maybe of giant size) than anterior lesions (Sincipital). Surgical outcomes are generally good.
