Diagnosing and Managing Androgen Insensitivity Syndrome

• 作者: Arya Zarif Agah Tashkand Cyril Kamadjou Edouard Hervé Moby Mpah Franklin Danki Sillong Kimassoum Rimtebaye Lamine Niang Mignagnal Kaboro Serigne Magueye Gueye
• 来源期刊: 泌尿学期刊(英文)
• 学科: 医学
• 关键词: GENITAL MALFORMATION ANDROGEN Genitoplasty VAGINOPLASTY CHROMOSOMAL SEX
• 年，卷（期）: mnxqkyw_2017,（11）
• 页码范围: 219-225
• 页数: 7页
• 分类号: R73

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.