Cloacal Exstrophy

Shannon Koehler, MD, Marc Levitt, MD, Casey M Calkins, MD
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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