The long-term management and outcomes of cloacal anomalies

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• 作者：M. Ashani Fernando ; Sarah M. Creighton ; Dan Wood
• 关键词：Cloacal anomalies ; Long ; term follow ; up ; Renal function ; Vaginoplasty ; Reconstruction
• 刊名：Pediatric Nephrology
• 出版年：2015
• 出版时间：May 2015
• 年：2015
• 卷：30
• 期：5
• 页码：759-765
• 全文大小：280 KB
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  Gillenwater, JY, Grayhack, JT eds. (2002) Adult and paediatric Urology. Lippincott, Williams and Wilkins, Philadelphia
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• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pediatrics
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1432-198X

文摘

Cloacal anomalies occur when failure of the urogenital septum to separate the cloacal membrane results in the urethra, vagina, rectum and anus opening into a single common channel. The reported incidence is 1:50,000 live births. Short-term paediatric outcomes of surgery are well reported and survival into adulthood is now usual, but long-term outcome data are less comprehensive. Chronic renal failure is reported to occur in 50 % of patients with cloacal anomalies, and 26-2 % (dependant on the length of the common channel) of patients experience urinary incontinence in adult life. Defaecation is normal in 53 % of patients, with some managed by methods other than surgery, including medication, washouts, stoma and antegrade continent enema. Gynaecological anomalies are common and can necessitate reconstructive surgery at adolescence for menstrual obstruction. No data are currently available on sexual function and little on the quality of life. Pregnancy is extremely rare and highly risky. Patient care should be provided by a multidisciplinary team with experience in managing these and other related complex congenital malformations. However, there is an urgent need for a well-planned, collaborative multicentre prospective study on the urological, gastrointestinal and gynaecological aspects of this rare group of complex conditions.