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23 - The gynaecology of the major genitourinary anomalies

from Part III - Management of specific disorders

Published online by Cambridge University Press:  04 May 2010

Christopher R. J. Woodhouse
Affiliation:
The Institute of Urology and the Hospital for Sick Children, Great Ormond St, London, UK
Adam H. Balen
Affiliation:
Leeds Teaching Hospitals, University Trust
Sarah M. Creighton
Affiliation:
University College London Hospitals
Melanie C. Davies
Affiliation:
University College London
Jane MacDougall
Affiliation:
Addenbrooke's Hospital, Cambridge
Richard Stanhope
Affiliation:
Great Ormond Street Hospital
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Summary

Introduction

There are three congenital genitourinary anomalies that have a serious impact on female sexual and reproductive function: exstrophy, spina bifida and the cloacal anomalies. It is true that spina bifida is primarily a neurological condition, but in practice it is the urological complications that dominate childhood and adolescence. The urologist usually manages the overall care.

Classical exstrophy has been recognized for about 2000 years and has been the subject of voluminous surgical literature since the middle of the last century. The results of exstrophy reconstruction, both functional and cosmetic, have improved considerably since the 1980s. The present adult women who were born with exstrophy would usually have had an early diversion and, if they wished, a continent reconstruction when such surgery became available. Few of them would have had a reconstruction that allowed them to void naturally and fewer still will have maintained that ability into their third decade (Woodhouse and Redgrave, 1996). Now, in specialist centres, primary reconstruction is undertaken in the first hours of life. Continence and spontaneous voiding are the norm; reconstruction with intestine and intermittent clean self-catheterization (ICSC) are sometimes needed.

With this general improvement has come the realization that exstrophic patients grow up and want to work, marry and have children. Exstrophy is an isolated anomaly in otherwise normal children. They grow up normally and, anecdotally, are intelligent and well-motivated adults.

Type
Chapter
Information
Paediatric and Adolescent Gynaecology
A Multidisciplinary Approach
, pp. 293 - 309
Publisher: Cambridge University Press
Print publication year: 2004

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