Pediatr Neurosurg. 2000 Aug;33(2):100-4.

Basal meningoencephalocele, anomaly of optic disc and panhypopituitarism in association with moyamoya disease.

Komiyama M¹, Yasui T, Sakamoto H, Fujita K, Sato T, Ota M, Sugita M.

Author information

1: Departments of Neurosurgery, Pediatric Neurosurgery, Pediatrics, Internal Medicine, Ophthalmology and Otorhinolaryngology, Osaka City General Hospital, Osaka, Japan. komiyama@japan-mail.com

Abstract

Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases.