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KMID : 0357919860200040470
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Korean Journal of Pathology
1986 Volume.20 No. 4 p.470 ~ p.474
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Male Pseudohermaphroditism Associated with Bochdalek Diaphragmatic Hernia
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Abstract
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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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KEYWORD
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