Disorders of sex development. Issue 12 (December 2016)

• Record Type: Journal Article
• Authors: Woodward, Mark; Roberts, Rebecca
• Is Part Of: Surgery. Volume 34:Issue 12(2016)
• Page Start: 633
• Page End: 638
• Publication Date: 2016-12
• Subjects: CAH -- congenital adrenal hyperplasia -- disorders of sex development -- DSD -- hypospadias -- intersex; Surgery -- Periodicals; Surgery -- Periodicals; Chirurgie -- Périodiques; Thérapeutique -- Périodiques; Electronic journals; 617
• DOI: 10.1016/j.mpsur.2016.09.005
• Languages: English
• ISSNs: 0263-9319

Abstract

Abstract: Disorders of sex development (DSD) occur in 1–2/10, 000 live births, with a specific molecular diagnosis only possible in 20% of cases. Presentation is usually at birth, and gender assignment must be avoided before review by an expert multidisciplinary team. Initial investigations allow a working diagnosis to be made within 48 hours. In 46, XY DSD, surgery may be necessary to correct hypospadias, reposition or remove undescended testes, and remove symptomatic Müllerian remnants. In 46, XX DSD, feminizing surgery is performed less frequently than in the past, but genitoplasty may still be indicated. Psychosocial support is required to promote positive adaptation as gender dissatisfaction can occur in certain conditions. Long-term outcome data are sparse.