Spheno-orbital encephalocele: A rare entity – A case report and review of literature

OBJECTIVE: To report a rare case of basal encephalocele (spheno-orbital encephalocele), managed successfully in our institute. This is one of the rarest type of encephaloceles with very little literature available. In this case, sphenoid dysplasia was not associated with type 1 Neurofibromatosis. SETTINGS: Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India. METHODS: A 22 year old male presented with a history of protrusion of right eye since the age of 7 years followed by progressive diminution of vision in the same eye for 5 months. MRI scan was suggestive of sphenoid dysplasia with herniation of right fronto-temporal lobe. The patient was operated upon with right frontal craniotomy with excision of encephalocele and repair of skull base. Subsequently, the patient underwent plastic surgery for facial skin remodeling. RESULTS: Patient's encephalocele reduced completely after excision and repair with good cosmetic results and no neurological deficits. There is no evidence of recurrence till recent follow-up. CONCLUSION: Basal encephaloceles are very rare. Complete excision of encephalocele with repair of the defect should be the aim to achieve cure.