Transethmoidal encephalocele: an unusual cause of pediatric nasal obstruction

Abstract

An encephalocele is a congenital malformation characterized by the herniation of brain content beyond the normal confines of the skull. Transethmoidal encephaloceles represent a rare subtype of anterior encephaloceles and consists in a protrusion of the brain content in the nasal cavity through a defect in the horizontal plate of the ethmoid bone. Clinically it usually manifests by upper airway obstruction. We report the case of an 8-month-old infant with a transethmoidal encephalocele revealed by a chronic nasal obstruction and snoring. The patient underwent left frontal craniotomy allowing repositioning of the herniation. Ethmoidal defect was repaired by a fascia lata graft. Post operative recovery was uneventful apart from a CSF rhinorrhea which spontaneously dried up in 3 days.