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[Female pseudohermaphroditism with a phallic urethra--a case report]
Hua Xi Yi Ke Da Xue Xue Bao = Journal of West China University of Medical Sciences = Huaxi Yike Daxue Xuebao
|June 1, 1992
Summary
Maternal virilizing adrenal tumors can cause female pseudohermaphroditism in infants. Early fetal androgen exposure leads to male-appearing genitalia in females, necessitating thorough diagnosis.
Area of Science:
- Endocrinology
- Pediatric Endocrinology
- Reproductive Endocrinology
Background:
- Female pseudohermaphroditism is a rare condition where an individual with XX chromosomes presents with ambiguous or male-like external genitalia.
- Maternal virilizing conditions, such as adrenal tumors, can expose a female fetus to excessive androgens during critical developmental periods.
Observation:
- This article reports a rare case of female pseudohermaphroditism with a penile urethra.
- The condition was attributed to a virilizing adrenal tumor in the mother, confirmed pathologically after surgical intervention.
Findings:
- Exposure of a female fetus to androgens before the eighth week of gestation can lead to the development of male external genitalia.
- Pathological examination confirmed the diagnosis post-operation.
Implications:
- It is crucial to investigate maternal virilizing tumors in cases of unexplained female pseudohermaphroditism.
- Phenotypic males with cryptorchidism born to virilized mothers require sex chromatin and chromosomal analysis to rule out pseudohermaphroditism.