A Case of Complete Androgen Insensitivity Syndrome with Bilateral Inguinal Gonads.

Kim, Jong In; Rhee, Jeong Ho; Eun, Myoung Hee

Case Report

Korean Journal of Obstetrics & Gynecology 1999;42(3):632-636.
Published online January 1, 2001.

A Case of Complete Androgen Insensitivity Syndrome with Bilateral Inguinal Gonads.

Jong In Kim, Jeong Ho Rhee, Myoung Hee Eun

Abstract

Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organ to the peripheral effect of androgen. Patient have a male karyotype(46,XY) and bilateral testes. Appearance of external genitalia depend on the degree of androgen insensitivity. In the complete form, external genitalia is normal female but in the incomplete form, external genitalia figure varies hom that of a virilized female to that of an undervirilized male with a short penis and hypospadia. The gonads are able to be located along the descending course of the testis during development. The usual presenting symptom in complete type is primary amenorrhea or inguinal mass, but in incomplete type, the patient mainly present with ambiguous genitalia. Recently we experienced one case of this syndmme and removed the bilateral gonads, so we report it with brief review of literatures.

Key Words: Androgen Insensitivity Syndrome