Congenital Adrenal Hyperplasia: A Critical Appraisal of the Evolution of Feminizing Genitoplasty and the Controversies Surrounding Gender Reassignment

 

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Abstract

Although congenital adrenal hyperplasia (CAH) is the most common cause of genital ambiguity, its surgical treatment remains controversial and often times difficult, especially in cases of high urethro-vaginal confluence. Many aspects are still uncertain, such as the timing of feminizing genitoplasty and type of surgical technique. The objective of this study is to provide a thorough review of the surgical management of children with CAH, discussing the evolution of feminizing genitoplasty and its different techniques, and also to critically appraise the available literature on the timing of surgical intervention and gender reassignment. Prospective long-term studies evaluating the results of modern feminizing genitoplasty techniques are needed as current evidence is based on outdated operations that are no longer used. To date, there have been no studies comparing early and delayed feminizing genitoplasty with regard to psychological outcomes. All families should be counseled regarding the controversies and treatment options, including the watchful waiting approach.

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Correspondence

Dr. Joao L. Pippi Salle

The Hospital for Sick Children

Urology

Toronto

Canada

Phone: 416/813 64 60

Fax: 416/813 64 61

Email: pippi.salle@sickkids.ca