Naso-ethmoidal encephalocele with bilateral orbital extension: report of a case in a western country

Abstract

Encephalocele is a rare congenital malformation of the central nervous system with protrusion of cranial content (meninges, brain, and ventricles in different combinations) beyond the normal confines of the skull. Anterior encephaloceles occur with a high frequency in Southeast Asia, while in the Western countries occipital encephaloceles prevail. The treatment of an anterior (naso-ethmoidal) encephalocele involves a neurosurgeon or a multidisciplinary team (neurosurgeon, maxillofacial surgeon, plastic surgeon, and ENT surgeon) dealing with craniofacial surgery. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures. The prognosis depends from the anatomical site, volume of neural contents, and the presence of coexisting malformations. We report the case of an Italian child suffering from a naso-ethmoidal encephalocele with bilateral orbital extension. The surgical treatment was performed in two steps. Sincipital encephalocele is a complex pathology without a unique standardized surgical treatment. Its low incidence in Western countries can make its management particularly tricky.