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      Treatment of the Enlarged Clitoris

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          Abstract

          Management of the enlarged clitoris, because of its import for sexual function, has been and remains one of the most controversial topics in pediatric urology. Early controversy surrounding clitoroplasty resulted from many factors including an incomplete understanding of clitoral anatomy and incorrect assumptions of the role of the clitoris in sexual function. With a better understanding of anatomy and function, procedures have evolved to preserve clitoral tissue, especially with respect to the neurovascular bundles. These changes have been made in an effort to preserve clitoral sensation and preserve orgasmic potential. It is the goal of this manuscript to describe the different procedures that have been developed for the surgical management of clitoromegally, with emphasis on the risks and benefits of each. Equally important to any discussion of such a sensitive topic is an understanding of long-term patient outcomes. As we will see, despite its importance, there has been a dearth of data in this regard. Future work in the arena of patient satisfaction will undoubtedly play a major role in directing our surgical approach.

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          Most cited references68

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          Congenital adrenal hyperplasia.

          Congenital adrenal hyperplasia (CAH) due to deficiency of 21-hydroxylase is a disorder of the adrenal cortex characterised by cortisol deficiency, with or without aldosterone deficiency, and androgen excess. Patients with the most severe form also have abnormalities of the adrenal medulla and epinephrine deficiency. The severe classic form occurs in one in 15,000 births worldwide, and the mild non-classic form is a common cause of hyperandrogenism. Neonatal screening for CAH and gene-specific prenatal diagnosis are now possible. Standard hormone replacement fails to achieve normal growth and development for many children with CAH, and adults can experience iatrogenic Cushing's syndrome, hyperandrogenism, infertility, or the development of the metabolic syndrome. This Seminar reviews the epidemiology, genetics, pathophysiology, diagnosis, and management of CAH, and provides an overview of clinical challenges and future therapies.
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            Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology.

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              Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia.

              In congenital adrenal hyperplasia (CAH) caused by different mutations, feminizing surgery is mostly performed in childhood, and many patients are lost to follow-up. A follow-up study on 62 CAH women aged 18-63 yr and 62 age-matched controls to correlate findings of both operative method and mutation was performed. Semistructured interviews were performed in cases and controls, as well as a gynecological examination in the cases. The results were correlated with disease-causing mutations and earlier surgical procedures if performed. The study was conducted at university hospital referral clinics. Gynecological examination in all cases correlated to previous surgery (n = 49), type of mutation, and questionnaire responses. Half of the CAH women claimed that the disease affected their sex life. The women were less satisfied with their genitals, whether operated or not. Clitoris size and functions were affected by the surgical method. Five women had a clinically evident vaginal stenosis on examination. However, almost half of patients experienced a narrow vagina. The overall psychosexual aspects of life were affected in these patients with later sexual debut, fewer pregnancies and children, and an increased incidence of homosexuality. These quality of life factors were correlated to the severity of the mutations. The overall quality of life in adult women with CAH is affected both by the type of mutation and operative procedure. Indications for clitoroplasty should be restrictive. Medical, surgical, and psychological treatment should be centralized.
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                Author and article information

                Contributors
                Journal
                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                2296-2360
                28 August 2017
                2017
                : 5
                : 125
                Affiliations
                [1] 1Pediatric Urology, Indiana University School of Medicine , Indianapolis, IN, United States
                Author notes

                Edited by: Ricardo González, Kinder-und Jugendkrankenhaus AUF DER BULT, Germany

                Reviewed by: Maria Marcela Bailez, Garrahan Childrens Hospital Buenos Aires, Argentina; Santiago Vallasciani, King Faisal Specialist Hospital & Research Centre, Saudi Arabia

                *Correspondence: Martin Kaefer, mkaefer@ 123456iupui.edu

                Specialty section: This article was submitted to Pediatric Urology, a section of the journal Frontiers in Pediatrics

                Article
                10.3389/fped.2017.00125
                5581357
                28894728
                869400b9-426b-47a8-8bf2-ce20ab9b34a7
                Copyright © 2017 Kaefer and Rink.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 30 March 2017
                : 09 May 2017
                Page count
                Figures: 12, Tables: 0, Equations: 0, References: 71, Pages: 11, Words: 8260
                Categories
                Pediatrics
                Review

                congenital adrenal hyperplasia,clitoromegally,clitoroplasty,disorders of sex development,history

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