Cloacal Exstrophy | Symptoms and Causes

How common is cloacal exstrophy?

Cloacal exstrophy is a very rare birth defect. It occurs once in every 250,000 births.

What are the components of cloacal exstrophy?

  • Omphalocele — Some of the abdominal organs protrude through an opening in the lower abdominal wall muscles in the area of the umbilical cord. A translucent membrane covers the protruding organs. The omphalocele may be small, with only a portion of the intestine protruding, or large, with most of the abdominal organs (including intestine, liver, and spleen) protruding outside of the body.

  • Cloacal exstrophy — The bladder is open in the front and the inner surface is exposed on the lower surface of the abdominal wall. The exposed bladder appears as a right and left half, with the large intestine (rectum/ hindgut) exposed and connected in the middle. This abnormal connection of the intestine and the bladder allows mixing of stool and urine.

  • Imperforate anus — The anus has not been formed, and the colon connects to the bladder as detailed above.

  • Spinal defects — These defects may either be major or minor. Many times children born with cloacal exstrophy are also born with varying degrees of spina bifida.

What causes cloacal exstrophy?

The cause of cloacal exstrophy is unknown. It does not appear that any medications or activities of the parents or expectant mother have any impact on the development of cloacal exstrophy. Based on our current knowledge, it is very unlikely that this condition can be prevented.