Korean J Obstet Gynecol.  2000 May;43(5):922-926.

A Case of Female Pseudohermaphroditism with m llerian agenesis, urinary tract malformations, and imperforate anus

Abstract

Special idiopathic female pseudohermaphroditism with urinary tract, m llerian duct, and lower gastrointestinal tract anomalies, in addition to masculinization of the external genitalia, is rare phenomenon. Masculinization of external genitalia and other anomalies occur in the absence of a recognized exposure to androgen or other teratogenic factors. We report a case of a female pseudohermaphroditism with m llerian agenesis, bilateral multicystic dysplastic kidney, urethral agenesis, left double ureter, rectovesical fistula, imperforate anus, single umbilical artery, detected after therapeutic abortion at 23+5 weeks of gestational age. The final diagnosis is based on autopsy.

Keyword

female pseudohermaphroditism; m llerian agenesis; urinary tract anomalies; imperforate anus

MeSH Terms

46, XX Disorders of Sex Development*
Abortion, Therapeutic
Anus, Imperforate*
Autopsy
Diagnosis
Female*
Fistula
Genitalia
Gestational Age
Humans
Lower Gastrointestinal Tract
Multicystic Dysplastic Kidney
Pregnancy
Single Umbilical Artery
Ureter
Urinary Tract*
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