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Issues in the Long-Term Management of Adolescents and Adults with DSD: Management of Gonads, Genital Reconstruction, and Late Presentation of the Undiagnosed DSD

  • Chapter
Transition and Lifelong Care in Congenital Urology

Part of the book series: Current Clinical Urology ((CCU))

Abstract

Disorders of sexual development (DSD) present a medical challenge on multiple levels. Prompt recognition of a disorder and appropriate diagnosis leading to individualized medical/surgical management are complex tasks. In older patients especially, these factors may potentiate the already significant psychological stress that has impacted the parents and family. Diagnostic and management controversies have existed since these conditions were first described. Today, as much as ever, the concept of what defines an individual’s sex is heavily debated, and hence have issues related to the timing and appropriateness of any interventions. In fact, in some conditions, therapeutic delay is now considered an alternative so that surgery, once commonplace in infancy and childhood, is now offered after puberty (e.g., gonadectomy in complete androgen insensitivity syndrome). As we gain more knowledge and our long-term experience with these conditions broadens, we recognize that there are many issues that persist or develop in the adolescent and adult period that require careful discussion and potential surgical intervention. Indeed, previously unrecognized cases of DSD may present after childhood, raising unique considerations. Thus, the surgeon who once dealt primarily with DSD in infancy must be cognizant of the ever-changing guidelines and opinions related to the various disorders and engage the adolescent and young adult in the options available and the informed consent process. From a surgical standpoint, one needs to be able to manage issues pertaining to the gonads and genitals (internal and external) in established as well later presentations of previously undiagnosed DSD.

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  1. Department of Pediatric Urology, Hospital for Sick Children, Toronto, ON, Canada

    Martin Koyle M.D., F.A.A.P., F.A.C.S., F.R.C.S.C., F.R.C.S. (Eng.) & Paul Bowlin M.D.

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  1. Martin Koyle M.D., F.A.A.P., F.A.C.S., F.R.C.S.C., F.R.C.S. (Eng.)
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Correspondence to Martin Koyle M.D., F.A.A.P., F.A.C.S., F.R.C.S.C., F.R.C.S. (Eng.) .

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  1. Center for Genitourinary Reconstruction, Glickman Urological and Kidney Institute, Cleveland, Ohio, USA

    Hadley M. Wood

  2. Adolescent and Reconstructive Urology, University College London Hospitals, London, United Kingdom

    Dan Wood

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Koyle, M., Bowlin, P. (2015). Issues in the Long-Term Management of Adolescents and Adults with DSD: Management of Gonads, Genital Reconstruction, and Late Presentation of the Undiagnosed DSD. In: Wood, H., Wood, D. (eds) Transition and Lifelong Care in Congenital Urology. Current Clinical Urology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-14042-1_7

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