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Cloacal Exstrophy

Shannon Koehler, MD, Marc Levitt, MD, Casey M Calkins, MD
Cloacal Exstrophy is a topic covered in the Pediatric Surgery NaT.

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Cloacal exstrophy (CE) is a rare congenital condition wherein the open bladder is divided in half by the cecal plate and is herniating through a lower midline abdominal wall defect. This condition is part of a spectrum of diseases referred to as the exstrophy-epispadias complex (EEC). The three most common conditions within this complex are epispadias, classic bladder exstrophy and cloacal exstrophy . Cloacal exstrophy is the most severe of these conditions - all of which are abdominal pelvic fusion abnormalities. Furthermore, CE is often associated with spinal dysraphism and when spinal defects are present, it may be referred to as the omphalocle exstrophy imperforate anus spinal defects (OEIS) complex.

Cloacal exstrophy has gone by several names since its description including ectopic cloaca, exstrophia splanchnica and vesicointestinal fissure [1]. It was first described in 1709 by Littre [2] but despite its relatively early characterization, the prognosis remained dismal for two and a half centuries. Repair was not attempted until 1900 [2] and the first successful repair was performed by Rickman in 1957 [1]. Even after successful repair was performed, malnutrition and sepsis remained problematic resulting in high mortality rates until the 1970s [3]. Since that time, the mortality rate has gradually declined to approximately ten percent and the current focus is on improving quality of life.

Content in this topic is referenced in SCORE Cloacal Exstrophy/Bladder Exstrophy overview

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Last updated: August 3, 2018


Koehler, Shannon, et al. "Cloacal Exstrophy." Pediatric Surgery NaT, American Pediatric Surgical Association, 2018. APSA Webapp, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829526/all/Cloacal_Exstrophy.
Koehler S, Levitt M, Calkins CM. Cloacal Exstrophy. In: Waldhausen J, Powell D, Hirschl R, eds. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829526/all/Cloacal_Exstrophy. Accessed August 20, 2019.
Koehler, S., Levitt, M., & Calkins, C. M. (2018). Cloacal Exstrophy. In Waldhausen, J., Powell, D., & Hirschl, R. (Eds.), Pediatric Surgery NaT. Available from https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829526/all/Cloacal_Exstrophy
Koehler S, Levitt M, Calkins CM. Cloacal Exstrophy [Internet]. In: Waldhausen J, Powell D, Hirschl R, editors. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. [cited 2019 August 20]. Available from: https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829526/all/Cloacal_Exstrophy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cloacal Exstrophy ID - 829526 A1 - Koehler,Shannon,MD AU - Levitt,Marc,MD AU - Calkins,Casey,MD ED - Waldhausen,John, ED - Powell,David, ED - Hirschl,Ron, Y1 - 2018/08/03/ BT - Pediatric Surgery NaT UR - https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829526/all/Cloacal_Exstrophy PB - American Pediatric Surgical Association DB - APSA Webapp DP - Unbound Medicine ER -