All issues > Volume 33(6); 1990

Original Article

J Korean Pediatr Soc. 1990;33(6):842-847. Published online June 30, 1990.

Transsphenoidal Meningoencephalocele in Association with Hypopituitarism ans Congenital Dysplastic Optic Disc: A Case Report.

Chang Gee Kang¹, Jung Wan You¹, Sung Chul Shin¹, Myung Goo Min¹, Duk Hi Kim¹, Jin Guk Kim², Oh Wong Kwon², Tae Sub Chung³

¹Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea
²Department of Ophthalmology, Yonsei University, College of Medicine, Seoul, Korea
³Department of Radiology, Yonsei University, College of Medicine, Seoul, Korea

Received: January 8, 1990;  Accepted: March 29, 1990.

Abstract

Transsphenoidal meningoencephalocele is congenital defects in the sphenoid bone through which meninges and brain tissue have herniated; the defect, located in the midline of the base of the skull, was first reported by Richter in 1813. The nine cases of transsphenoidal meningoencephalocele have been reported. Patients with transsphenoidal meningoencephalocele also may have other midline craniocerebral and midfacial anomalies, including additional meningoencephalocele hypertelorism, cleft palate, and abnormalities of the optic nerve and optic disc. Because of the sella turcica in sphenoid bone is usually affected, the hypothalamic-pituitary dysfunction has naturally been occured. We have experienced a case of transsphenoidal meningoencephalocele in associated with hypopituitarism and congenital dysplastic optic disc. The diagnosis was established by clinical, combined pituitary function test and radiologic findings A brief review of literature was made.

Keywords :Transsphenoidal lmeningoencephalocele, Hypopituitarism, Congenital dysplastic optic disc