This is a rare congenital malformation that can be diagnosed antenatally. The pattern of inheritance is unknown due to the small numbers 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.

Incidence:

This is a rare disorder and is found in one in 400,000 births.

Inheritance:

Unknown. Too few patients to make adequate determinations.

Diagnosis:

Can easily be made prenatally as both the bladder and intestine are open anteriorly and oftentimes the spine is open posteriorly.

Treatment:

Modern staged repair has been utilized in the treatment of cloacal exstrophy. Initial assessment of the neonate should focus on stabilizating the medical status and determining if associated anomalies make reconstruction ethically advisable.

• Initial Closure: 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 simply brought together, and a colostomy performed. Most patients are brought up as females because of the extent of the deformity of the penis. In these patients, the testes are removed.
  
  
• Continence Repair: 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 who have a small bladder size.
  

Experience at the Johns Hopkins Hospital indicate that 72-75% of patients are free of urine leakage following reconstruction by the above mentioned stages. The deformity of the penis was corrected to the satisfaction of the patient and family in most instances. This, however, requires dedicated and intensive treatment and follow-up by the patients and the medical personnel caring for them.

1. 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.
   
2. 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.