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      Long-term Follow-up after Feminizing Genital Reconstruction in Patients with Ambiguous Genitalia and High Vaginal Confluence

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          Abstract

          We evaluated the long-term results of feminizing genital reconstruction in patients with genital ambiguity with high vaginal confluence. The medical records of 10 consecutive patients with ambiguous genitalia and high vaginal confluence who underwent feminizing genital reconstruction from 1996 to 2007 were reviewed. Seven patients had congenital adrenal hyperplasia, one had mixed gonadal dysgenesis, one had partial androgen insensitivity, and one had 5-alpha reductase deficiency syndrome. Median age at operation was 21 months (range, 2-47 months). Median follow up was 7.7 yr. Of the six patients who underwent feminizing genital reconstruction with the Gonzalez method, three had good results. Of the other three patients, one had a urethrovaginal fistula and underwent fistula repair 9 yr after, one had distal vaginal stenosis and underwent revision vaginoplasty 9 yr after, and one had a urethrovaginal fistula and urethral stricture. The patient with urogenital mobilization had persistent urogenital sinus. Feminizing genitoplasty using the Gonzalez method showed good long-term results in patients with ambiguous genitalia and Congenital adrenal hyperplasia. The procedure is less invasive than other approaches and results in excellent cosmetic outcomes; and complications can be corrected by relatively simple procedures.

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          Most cited references18

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          Total urogenital mobilization--an easier way to repair cloacas.

          The surgical treatment of persistent cloaca is a serious challenge. The operation is technically difficult and the final results for urinary and fecal function are far from excellent. The repair of a cloaca includes, among other maneuvers, the separation of the vagina from the urinary tract. This step is a serious technical challenge and is very time consuming. Devascularization of these structures is the main source of complications such as urethro-vaginal fistula, vaginal stricture, and acquired vaginal atresia. To avoid these complications and to facilitate the cloacal repair, a new technical variation using total urogenital mobilization was performed in 11 patients. In this procedure, after the rectum is separated from the vagina, both the urethra and the vagina are mobilized together as a single unit. The surgical time spent during the reconstruction was reduced by approximately 70%. All patients recovered well from the operations and have been followed up for 1 to 14 months. The blood supply of the vagina and urethra in all cases remained excellent. No patient developed urethrovaginal fistula, vaginal stricture, or acquired vaginal atresia. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. Although this maneuver may not render better urinary or fecal control, the urethra is more accessible for catheterization. These preliminary results suggest that the total urogenital mobilization maneuver provides a definite technical advance in the repair of cloaca malformations.
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            A new 1-stage procedure for clitorovaginoplasty in severely masculinized female pseudohermaphrodites.

            A new 1-stage procedure for clitorovaginoplasty in severely masculinized female pseudohermaphrodites is presented. The technique consists of clitoral reduction with preservation of the neurovascular supply to the glans and vaginal formation by joining the inverted penile skin and the divided distal "urethra" (urogenital sinus) to form a cylinder that is then inserted into the perineum and anastomosed to the true vagina. For high and small vaginas Monfort's transtrigonal approach is suggested. This operation, 4 of which have been performed to date, can be done at any age, and it offers excellent cosmetic and functional results.
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              Subtunical total reduction clitoroplasty: a safe modification of existing techniques.

              Subtunical resection of hypertrophied cavernous tissue of the enlarged clitoris allows for maintenance of an intact blood supply to the glans and is an alternative safe method of diminishing shaft and glans size.
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                Author and article information

                Journal
                J Korean Med Sci
                JKMS
                Journal of Korean Medical Science
                The Korean Academy of Medical Sciences
                1011-8934
                1598-6357
                March 2011
                25 February 2011
                : 26
                : 3
                : 399-403
                Affiliations
                [1 ]Department of Urology, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea.
                [2 ]Department of Urology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
                Author notes
                Address for Correspondence: Kun Suk Kim, MD. Department of Urology, Asan Medical Center, University of Ulsan College of Medicine, 86 Asanbyeongwon-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82.2-3010-3736, Fax: +82.2-477-8928, kskim2@ 123456amc.seoul.kr
                Article
                10.3346/jkms.2011.26.3.399
                3051088
                21394309
                20e6106b-daac-4398-b660-3f60b03c80ec
                © 2011 The Korean Academy of Medical Sciences.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 16 September 2010
                : 10 January 2011
                Categories
                Original Article
                Urology

                Medicine
                feminization,disorders of sex development,adrenal hyperplasia, congenital
                Medicine
                feminization, disorders of sex development, adrenal hyperplasia, congenital

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