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The long-term management and outcomes of cloacal anomalies.

Fernando MA, Creighton SM, Wood D - Pediatr. Nephrol. (2014)

Bottom Line: 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.Pregnancy is extremely rare and highly risky.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University College London Hospitals, 250 Euston Road, London, NW1 2PG, UK.

ABSTRACT
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 26-72 % (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.

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Related in: MedlinePlus

Photograph of an adult perineum in a female patient with a previously operated cloacal anomaly
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Fig1: Photograph of an adult perineum in a female patient with a previously operated cloacal anomaly

Mentions: Cloacal anomalies (persistent cloaca) are abnormalities of the urogenital sinus and anorectum; to date, their aetiology is still unknown (Fig. 1). These conditions are rare, although the true incidence is difficult to ascertain due to differences in classification and the inclusion of other rectal anomalies in some estimates. However, it is thought that the incidence of cloacal anomalies worldwide is approximately 1:50,000 live births [1].Fig. 1


The long-term management and outcomes of cloacal anomalies.

Fernando MA, Creighton SM, Wood D - Pediatr. Nephrol. (2014)

Photograph of an adult perineum in a female patient with a previously operated cloacal anomaly
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4372671&req=5

Fig1: Photograph of an adult perineum in a female patient with a previously operated cloacal anomaly
Mentions: Cloacal anomalies (persistent cloaca) are abnormalities of the urogenital sinus and anorectum; to date, their aetiology is still unknown (Fig. 1). These conditions are rare, although the true incidence is difficult to ascertain due to differences in classification and the inclusion of other rectal anomalies in some estimates. However, it is thought that the incidence of cloacal anomalies worldwide is approximately 1:50,000 live births [1].Fig. 1

Bottom Line: 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.Pregnancy is extremely rare and highly risky.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University College London Hospitals, 250 Euston Road, London, NW1 2PG, UK.

ABSTRACT
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 26-72 % (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.

Show MeSH
Related in: MedlinePlus