Cloacal Exstrophy
What is cloacal exstrophy?
Cloacal exstrophy is a rare congenital malformation that can be diagnosed antenatally. The pattern of inheritance is unknown due to the small number of patients. The hindgut and cecum is exstrophied between two halves of the bladder. There is prolapse of the ileum and the handgut is blind ending. There is significant diastasis of the pubic symphysis and the phallus is also separated into two halves along with the scrotum. This is a rare disorder and is found in one in 400,000 births.
Diagnosis
Diagnosis can easily be made prenatally as both the bladder and intestine are open anteriorly and oftentimes the spine is open posteriorly.
Associated conditions: Abnormalities of the kidneys are more frequently noted. The testes may not come down into the scrotum (sac). In the female child, the vagina may be absent, and the clitoris is split into two halves. A higher incidence of renal anomalies is seen in patients with cloacal exstrophy. Genital anomalies include absent or bifid phallus. Vaginal absence may also be noted. Anomalies of the extremities, cardiovascular system, bowel, vertebrae and diaphragm also have been seen.
Treatment
Modern staged repair has been utilized in the treatment of cloacal exstrophy. Initial assessment of the neonate should focus on stabilizing the medical status and determining if associated anomalies make reconstruction ethically advisable.
A primary repair is done soon after birth if the child is robust and healthy. The bladder is closed and the pelvic bones are brought together into their normal ring structure. The bowel is brought out to a stoma (bag) on the wall of the abdomen. If the baby cannot tolerate a procedure of this magnitude, the bowel is removed from the abdominal wall, the bladder halves are simply brought together and a colostomy is performed.
Continence procedure: If bladder closure is delayed because of associated defects, closure is performed at one year of age. At four to seven years of age, a procedure to establish urinary continence is performed. This involves the reconstruction of the outlet of the bladder to prevent urine leakage. The ureters (tubes from the kidneys to the bladder) are also repositioned. The bowel may be used to further increase the size of the bladder in those patients that have a small bladder size.
Adolescent repair is performed at the time of adolescence. This involves the construction of a vagina or the enlargement of the vagina in those children with a narrow vagina.
The treatment protocols and the timing of procedure requires individualization based on the extent of the defect noted. The use of multidisciplinary approach at a large center is the key to good functional and cosmetic results with decreased morbidity.
Follow-up
Follow-up is intensive and long-term. Most patients should be referred to a large center where facilities are available for the care of complex birth defects.
References
John P. Gearhart, Robert D. Jeffs: Exstrophy of the Bladder, Epispadias and other Bladder Anomalies in Campbell's Urology, Sixth Edition. Eds. Walsh PC, Retik AB, Stamey TA, Darracott Vaughan E, Jr., WB Saunders Co. Vol. 2 1772-1821.
John P. Gearhart: The bladder exstrophy-epispadias complex. In: Pediatric Urology. Es Gearhart JP, Rink RR, and Mouriquand P. Saunders, Philadelphia. Chapter 32, p 511-546.
