529 MANAGEMENT OF CHILDREN WITH AMBIGUOUS GENITALIA USING A “DISORDERS OF SEX DEVELOPMENT (DSD) TEAM”

Abstract

You have accessJournal of UrologyPediatrics: Congenital Anomalies - Lower Urinary Tract & Genitalia1 Apr 2010529 MANAGEMENT OF CHILDREN WITH AMBIGUOUS GENITALIA USING A “DISORDERS OF SEX DEVELOPMENT (DSD) TEAM” Saul Greenfield, Tom Mazur, Glinianski Michal, and Teresa Quattrin Saul GreenfieldSaul Greenfield More articles by this author , Tom MazurTom Mazur More articles by this author , Glinianski MichalGlinianski Michal More articles by this author , and Teresa QuattrinTeresa Quattrin More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2010.02.728AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Controversy continues regarding the proper management of children born with DSD. Since 1985, a multidisciplinary “DSD Team” has been utilized to manage these children. Urology, endocrinology and psychology records were reviewed regarding surgical procedures, hormonal treatment and gender development. METHODS The combined data bases from the urology, endocrinology and psychology departments were reviewed from 1985 to 2008. Surgical, medical and psychological data were tabulated. RESULTS 14 patients were identified: 3-congenital adrenal hyperplasia (CAH), 4-true hermaphrodites (TH), 4-mixed gonadal dysgenesis (MGD), 1-androgen insensitivity syndrome (AIS), 1 with severe hypospadias, 1 with 46Xp+Y (sex reversal). Mean follow up was 12.5 years (4 to 25 yrs). 8 were reared as girls, 6 as boys. All 3 CAH were gender assigned as girls&em;1 has undergone clitoral reduction and 1 has had vaginoplasty. All are well managed on medical therapy. Of the 4 MGD patients, all were gender assigned as boys and underwent hypospadias repair and removal of mullerian ductal (MD) structures and ovarian issue. 3 are on androgen supplementation. Three of the four with TH were assigned and reared as boys and underwent hypospadias repair with removal of inappropriate gonadal and MD structures. The one assigned and reared female is on estrogen and undergoing vaginal dilatation. The 1 with AIS was reared female. The 1 with sex reversal (46Xp+Y) was assigned female. The 1 severe hypospadias patient has undergone repair and is reared male. Current psychosexual assessment indicates gender identity concordant with initial gender assignment in all 14. Furthermore, no gender dysphoria was identified in any patient. Sexual orientation is heterosexual in 6 (3M, 3F) and 4 are sexually active (2M, 2F). The remaining 8 were too young to assess sexual orientation. CONCLUSIONS Using a team approach, appropriate gender assignment can be made shortly after diagnosis, as suggested by the current evidence of gender development in these individuals. Ongoing medical, surgical and psychological interventions will permit the team to continually assess psychosexual development as well as quality of life for both patient and family. Buffalo, NY© 2010 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 183Issue 4SApril 2010Page: e209 Advertisement Copyright & Permissions© 2010 by American Urological Association Education and Research, Inc.MetricsAuthor Information Saul Greenfield More articles by this author Tom Mazur More articles by this author Glinianski Michal More articles by this author Teresa Quattrin More articles by this author Expand All Advertisement Advertisement PDF DownloadLoading ...