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A Case of Congenital Hypopituitarism Presenting Ectopic Pituitary Gland and Pituitary Stalk Transection Identified by Magnetic Resonance Imaging

Journal of the Korean Pediatric Society 1992;35(6):809-815.
Published online June 15, 1992.
A Case of Congenital Hypopituitarism Presenting Ectopic Pituitary Gland and Pituitary Stalk Transection Identified by Magnetic Resonance Imaging
Do Seong Kim1, Kyung Eun Oh1, Duk Hi Kim1, Myung Joon Kim2
1Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
2Department of Radiology, College of Medicine, Yonsei University, Seoul, Korea
자기공명영상상 이상 소견을 보인 선천성 뇌하수체기능저하증 1례
김호성1, 오경은1, 김덕희1, 김명준2
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 방사선과학교실
Abstract
The syndrome of congenital hypopitutarism is a rare but well recognized entity. The typical clinical picture consists of presentation in infanncy with hypoglycemia, microgenitalia, and evidence of multiple anterior pituitary hormone deficiencies, but not diabetes inspidus. Recently, magnetic resonance imaging has disclosed a consistent abnormality in many patients with congenital hypopituitarism as well as idiopathic hypopituitarism characterized by an adenohypophysis of varying volume, attenuation or transection of the pituitary stalk, absence of the usual intrasellar location of the high intensity signal of the posteior pituitary, and the presence of such an image at or below the median eminence of the hypothalamus, considered an ectopic neurohypophysis. We have recently seen a case of congenital hypopituitarism in a 34/12-year-old male patient with microgenitalia and short stature. The endocrinological studies revealed growth hormone and gonadotropin-releasing hormone deficiencies. The magnetic resonance imaging showed characteristic findings of congenital hypopituitarism. The patient was followed-up with growth hormone therapy.
Key Words: Congenital hypopituitarism, Magnetic resonance imaging, Ectopic posterior pituitary gland


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