Diagnosing and Managing Androgen Insensitivity Syndrome  

作　　者：Kimassoum Rimtebaye Edouard Hervé Moby Mpah Cyril Kamadjou Arya Zarif Agah Tashkand Franklin Danki Sillong Mignagnal Kaboro Lamine Niang Serigne Magueye Gueye 

机构地区：[1]General Referal National Hospital of N’Djamena, N’Djamena, Chad [2]Laquintinie Hospital of Douala, Douala, Cameroon [3]Protestant Hospital of N’Gaoundere, N’Gaoundere, Cameroon [4]Grand Yoff, General Hospital of Dakar, Dakar, Senegal

出　　处：《Open Journal of Urology》2017年第11期219-225,共7页泌尿学期刊（英文）

摘　　要：Introduction: Androgen insensitivity syndrome is a rare congenital abnormality of genital organs revealing a female phenotype in a person with XY chromosomes. The aim of our work is to describe the clinical aspects, to determine the chromosomal sex and to report our therapeutic management. Observation: A 28-year-old woman with female phenotype, feminine voice, normal breast development, normal underarms and pubic hair, absence of menstruation, fusion of small and large vaginal lips leaving a small pertuis to serve as urethral meatus. On the dorsal surface of the large, left lip was a mass the size of a date. Hormonal balance was normal. A feminizing genitoplasty was performed as well as excision of the mass. Histological analysis of the mass concluded that it was a feminizing testicular. Conclusion: Androgen insensitivity syndrome is a rare abnormality of the genitals. Multidisciplinary management is essential both for the designation of the breeding sex and feminizing genitoplasty.Introduction: Androgen insensitivity syndrome is a rare congenital abnormality of genital organs revealing a female phenotype in a person with XY chromosomes. The aim of our work is to describe the clinical aspects, to determine the chromosomal sex and to report our therapeutic management. Observation: A 28-year-old woman with female phenotype, feminine voice, normal breast development, normal underarms and pubic hair, absence of menstruation, fusion of small and large vaginal lips leaving a small pertuis to serve as urethral meatus. On the dorsal surface of the large, left lip was a mass the size of a date. Hormonal balance was normal. A feminizing genitoplasty was performed as well as excision of the mass. Histological analysis of the mass concluded that it was a feminizing testicular. Conclusion: Androgen insensitivity syndrome is a rare abnormality of the genitals. Multidisciplinary management is essential both for the designation of the breeding sex and feminizing genitoplasty.

关 键 词：GENITAL MALFORMATION ANDROGEN Genitoplasty VAGINOPLASTY CHROMOSOMAL SEX 

分 类 号：R73[医药卫生—肿瘤]