真性半陰陽の1例

Abstract

The patient was a two years and seven months old, who was legally a female. She visited our University Hospital with the chief complaint of ambiguous genitalia. The hypertrophied clitoris was 13 mm in length and 11 mm in diameter. The external urethral orifice and vagina were observed separately. The testis-like mass was palpable in the right labium. She had a karyotype of 46XX . The Urinary excretion of 17 KS and pregnanetriol and serum 17 alpha-hydroxyprogesterone were within the normal range. Vaginography showed the vagina, uterus and left fallopian tube. At exploratory laparotomy, we found the uterus and left fallopian tube; the left and right gonads were in the intraperitoneal cavity and the inguinal canal, respectively. To build female characteristics, clitoroplasty was performed, the right gonad was removed the testis-like portion of the left gonad was resected, and the ovary-like portion in the left gonad was biopsied. Both gonads were confirmed to be ovotestis histologically.