Atypical Presentation of Frontoethmoidal Encephaloceles in a Child - A Case Report

Abstract

Encephalocele is a form of neural tube defect affecting many children, more common in the occipital regions. The frontoethmoidal encephaloceles (FEE) are rare and the aetiology is unclear since it varies genetically and is linked to multiple reasons. The child with FEE mainly presents with swelling over the bridge of the nose or inner canthus of an eye since birth, with varying degrees of hypertelorism. Here we report a child who has facial asymmetry with FEE. Encephalocele is a defect in the calvarium that causes the cranial contents to protrude outside the natural limits of the skull. It can have meninges (meningocele), brain matter and meninges (meningoencephalocele), or communication with the ventricles. Suwanwela and Suwanwela defined encephaloceles based on the location and form of skull defect as occipital encephaloceles, cranial vault encephaloceles, frontoethmoidal encephaloceles, and basal encephaloceles. The internal skull defect in frontoethmoidal encephalocele (FEE) is in the midline, but the external skull defect may vary in the facial bony structure. 1–3 It is also classified into three subtypes: nasofrontal (NF), naso-ethmoidal (NE), and naso-orbital (NO). These swellings are classified as sessile or pedunculated, and their palpability ranges from robust and rigid to soft and cystic. We present a case of an infant with frontoethmoidal encephaloceles as well as a facial deformity.