Long-term outcome of genital reconstruction of Middle Eastern women with congenital adrenal hyperplasia

Abstract

Objectives: There is a paucity of data on the long-term outcome of genital reconstruction of female children with congenital adrenal hyperplasia (CAH) as they become adult women. We report on the surgical outcome general condition and marriage status. Materials and Methods: We reviewed the medical records of women 20 years or older with CAH who had genital reconstruction. We interviewed married patients utilizing the female sexual function index (FSFI-6) questionnaire and compared them to age-matched controls. Results : We identified 43 women with CAH with a median age of 24.2 ± 3.9 years and a median follow-up of 23.4 ± 4.6 years. Salt wasting and the severity of virilization affected most patients, parents were commonly cousins, children were reared as boys for a protracted period and surgical reconstruction was usually complex. Only five women had married. Compared with single women, married women had significantly more frequent normal menses, emergency hospital admissions and number of repeated reconstructive surgery. There was no significant difference in FSFI score between patients and controls. Four women conceived and three gave birth to one healthy child. There was no significant difference in the number of children between patients and controls. Conclusions: CAH has a significant impact on adult women in our region. Most of the patients remain single. Few women get married and these are able to lead a nearly normal sexual life and give birth to healthy children.

Keywords

• Adrenal hyperplasia
• congenital
• female urogenital diseases
• fertility
• follow-up studies
• reconstructive surgical procedures