Sphenoid cranial base defects in siblings presenting with cerebrospinal fluid leak

Two sisters presented to our medical center with nontraumatic cerebrospinal fluid (CSF) fistulas from left sphenoid sinocranial junction defects. One sister had recurrent meningitis over a 20-year period that prompted a skull base evaluation. Four years later, her younger sister presented with profuse CSF rhinorrhea. Transethmoid sphenoidotomy with sinus obliteration and lumbar-subarachnoid temporary CSF diversion successfully treated one sister, while the other required reoperation and permanent lumbar-peritoneal shunting. In both cases the skull base defect was identically located in the posterolateral left sphenoid sinus. Embryological considerations, evaluation, and management are presented.