What is the pathophysiology of cloacal exstrophy?

Updated: Nov 05, 2019
  • Author: James G Glasser, MD, MA, FACS; Chief Editor: Dharmendra J Nimavat, MD, FAAP  more...
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Answer

The urorectal septum divides the cloaca into the urogenital sinus and the rectum. Defective enfolding of the embryo's caudal pole, and deficient incorporation of the yolk sac and allantois into the urogenital sinus, leads to malformation of the external genitalia.

The cloaca persists in the absence of mesodermal ingrowth. Differentiation of the genitourinary system and hindgut are arrested, and development of the lower abdominal wall is thwarted.

Cloacal exstrophy is associated with mutations in the homeobox genes. (See the following images.)

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). This photograph shows cloacal exstrophy in an infant.
Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Note the bifid genitalia in this infant with cloacal exstrophy.

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