A rare case report of frontoethmoidal encephalocele in a neonate

Abstract

The protrusion of cranial contents via a skull defect is known as an encephalocele. Unlike western countries where occipital encephalocele dominates, anterior encephaloceles are the most common types of encephalocele in Southeast Asia, parts of Russia, and Central Africa. We present the clinical presentation and surgical management of an 8-day-old infant with frontoethmoidal encephalocele. An 8-day-old neonate born to a 24-year-old mother with no antenatal follow-up was referred with a compliant of frontonasal swelling which was present since birth. Physical examination of the neonate revealed a cystic lobulated swelling over the upper edge of the nasal bridge. Computed tomography imaging showed an anterior skull defect, resulting in frontonasal meningoencephalocele. The patient underwent a one-stage operation, which combined nasal-coronal approach with frontal craniotomy, to remove the dysplastic tissue and reconstruct the defect. The patient had successful recovery period with acceptable cosmesis. We presented a case of frontoethmoidal encephalocele, which is rare in western countries but whose incidence is relatively higher in African countries. Perinatal care is necessary for prevention and early detection of such cases. Direct surgical repair is still the main mode of intervention.