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

Kim, Yoon Sook; Kim, Sang Sool; Kim, Hyung Il; Min, Byung Jo; Park, Mi Young; Lee, Kyung Soon

Case Report

Korean Journal of Obstetrics & Gynecology 2000;43(5):922-926.
Published online January 1, 2001.

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

Yoon Sook Kim, Sang Sool Kim, Hyung Il Kim, Byung Jo Min, Mi Young Park, Kyung Soon Lee

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.

Key Words: female pseudohermaphroditism, m llerian agenesis, urinary tract anomalies, imperforate anus