| A Case of Severe Pituitary Dwarfism due to Agenesis of Anterior Pituitary Gland with Pituitary Stalk Transection |
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Myoung Ju Yoo1, Dong Ki Han1, Jeh Hoon Shin1, In Jun Seul1, Seung Ro Lee2 |
1Department of Pediatrics, Han yang University College of Medicine, Seoul, Korea
2Department of Radiology, Han yang University College of Medicine, Seoul, Korea |
| 뇌하수체 전엽 무형성에서 기인한 저신장 1례 |
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유명주1, 한동기1, 신재훈1, 설인준1, 이승로2 |
1한양대학교 의과대학 소아과학교실
2한양대학교 의과대학 방사선과학교실 |
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| Abstract |
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We experienced one case of severe pituitary dwarfism in a 10 years old female girl.
Magnetic resonance image(MRI) revealed transection of the pituitary stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland.
The serum Growth Hormone(GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH.
The morning cortisol concentration and serum T4 concentration were decreased below the normal range.
These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI.
Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary stalk.
We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature. |
| Key Words:
Pituitary dwarfism, MRI, Pituitary agenesis |
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