Bochdalek 탈장을 수반한 남성 가성반음양증
Male Pseudohermaphroditism Associated with Bochdalek Diaphragmatic Hernia
유은실, 지제근, 한준구,
소속 상세정보
유은실 ( )
서울대학교 의과대학 병리학교실
지제근 ( )
서울대학교 의과대학 병리학교실
한준구 ( )
서울대학교 의과대학 병리학교실
KMID : 0357919860200040470
Abstract
An autopsy case of male pseudohermaphrodite associated with Bochdalek diaphragmatic
hernia is reported. This newborn infant showed a large clitoris and hypospadic urethra
which was the only opening in the perineum except for the anus. There were
urethrovaginal fistula and uterus didelphys. The adrenals were slightly larger than
normal, but microscopically did not show any features of cortical hyperplasia. Well
formed testes were found and were histologically unremarkable. In addition, Bochdalek
type of diaphragmatic hernia was present, associated with severe left lung hypoplasia
and massively herniated abdominal organs into the chest cavity, shifting the
mediastinum considerably and eventually led to death in a few minutes after birth.
The pathogenesis of genital anomaly and diaphragmatin hernia is briefly discussed and
it is suggested that this type of genital abnormality might be due to defect of testicular
Mullerian inhibitor factor and can be categorized as incomplete male
pseudohermaphroditism.
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