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

Journal of the Korean Pediatric Society 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
하수체 기능 저하증과 선천성 안저이형성이 동반된 경접형골 수막뇌류 1례
강창기1, 유정완1, 신성철1, 민명구1, 김덕희1, 김진국2, 권오웅2, 정태섭3
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 안과학교실
3연세대학교 의과대학 방사선과학교실
Received: 8 January 1990   • Accepted: 29 March 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.
Key Words: Transsphenoidal lmeningoencephalocele, Hypopituitarism, Congenital dysplastic optic disc


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