Cloacal Malformation/Cloacal Exstrophy

Cloacal exstrophy is a complex condition that involves the genitourinary tract, the gastrointestinal tract, the musculoskeletal system and often neurologic system. These babies are referred to as having the OEIS complex when they have an omphalocele, imperforate anus and spinal defects with cloacal exstrophy. Omphalocele is where abdominal organs protrude through the abdominal wall where the umbilicus lies. Imperforate anus is where the opening for the rectum is not formed. Spinal defects can vary in degree. Boys often have a shortened penis that is split in half. In girls, the clitoris is split in half.

Cloacal exstrophy can be detected on prenatal ultrasound when there is the identification of a lower abdominal wall defect, a spinal cord defect and the bladder is not found. If not diagnosed prenatally, it is identified immediately after birth. A plastic wrap is placed on the exposed bladder halves that are separated by exposed bowel to protect it. A whole team of specialists will be involved in your child’s care. Surgical reconstruction is often done in stages and individualized to the needs of the child. Within the first few weeks of life, the omphalocele is closed, and the exposed bowel is made into a tube and brought out to the skin (colostomy) so that stool can eliminate through this. The bladder halves may be closed and placed in the pelvis or brought together and converted to a bladder exstrophy to allow for growth and closure in the future months. Following surgery, the baby is placed in a hip cast for about four weeks. Over the first year of life, the spine will be addressed, and the colostomy closed with a pull-through procedure, allowing the child to pass stool through the rectum. Surgical care is carefully planned individualized to the needs of the child.

Children with cloacal exstrophy require long-term follow up with a multidisciplinary approach. The urologic concerns that are addressed include the ability to store urine (continence), backflow of urine to the kidney (vesicoureteral reflux), ensure kidneys are growing, optimize bladder function and maximize external genital function and appearance.

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