Glial Heterotopia of Sphenoid in Association with a Patent Sternberg's Canal presenting with Meningitis

Abstract

ABSTRACT Ectopic glial tissue, presenting at various sites of body is a rare embryonic developmental anomaly. Very few cases are reported in adults, most being incidentally detected, revealed only by histopathological findings. One of the rarest locations is the sphenoid sinus. We present here the case of a middle aged female, presenting with spontaneous cerebrospinal fluid (CSF) rhinorrhea and meningitis. With the radiological finding of a soft tissue density in sphenoid sinus with erosion of left temporal skull base, she underwent lateral craniotomy, with excision of the mass and middle cranial fossa floor repair. Histopathology revealed a heterotopic glial tissue. Peroperative finding of a well-defined defect in the lateral wall of an extensively pneumatized sphenoid showed typical features of a patent Sternberg's canal. This case could be a living proof for the existence of this rare developmental anomaly. A persisting Sternberg's canal should be considered as the source of spontaneous CSF leaks with or without lesions in sphenoid sinuses with extensive lateral pneumatization. Most of the cases may be treated with an extended endoscopic approach. Extreme lateral lesions like this would benefit most from a craniotomy approach for proper closure of skullbase defect. Three years after successful surgery, she remains symptom free.