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Get the facts. And the help you need.

Cloacal Exstrophy

Sometimes factors can occasionally interfere with bladder development, as is the case for children with cloacal exstrophy. If your newborn has been diagnosed with this condition, what can you expect? The following information should help you talk to your child's doctor if he/she has been diagnosed with cloacal exstrophy.

What is cloacal exstrophy?

This is the most severe birth defect in the exstrophy-epispadias complex. A child with this condition will have the bladder and a portion of the intestines, exposed outside the abdomen, with the bony pelvis open like a book. In males the penis is either flat and short or sometimes split. In females the clitoris is split and there may be two vaginal openings. Also, frequently the intestine is short and the anus is not open. There is a high association with other birth defects, especially spina bifida, which occurs in up to 75 percent of cases. Omphalocele, a defect of the abdominal wall in the region of the umbilicus, is also common as are kidney abnormalities.

How often does it occur?

It is rare, occurring in approximately one in every 250,000 births and is slightly more common in males than females.

What caused this condition?

There is no known cause but it is also very unlikely that anything could have been done to prevent it.

How is it diagnosed?

Frequently it can be detected before birth during a routine sonogram. Nonetheless, this condition will be obvious at birth.

What is the treatment?

Surgical reconstruction is undertaken when the child is medically stable. The surgery is staged, but the schedule of surgery is very dependent on the individual child. The first surgical consideration is repair of any coexistent spinal abnormality, and perhaps repair of a large omphalocele. Once the child has recovered sufficiently from this, the gastrointestinal tract is then treated. A significant number of cases require a stoma because the colon is not normal, and the anus is not formed. Closure of the bladder and reconstruction of the genitalia are similar to that for classic exstrophy, although the procedure is sometimes staged because the pelvic bones are widely separated. In select cases the abdominal wall and genitourinary system can be repaired at the same time as the bowel. For a successful closure, a pelvic osteotomy (cutting the bones to allow the pelvis to close more easily) is mandatory. Achieving eventual continence almost always involves bladder reconstruction and using a catheter.

What can be expected after treatment?

The management of cloacal exstrophy has advanced to provide great improvement in the quality of life of affected children. With advances in pediatric anesthesia and infant nutrition the survival rate in the newborn is high and the incidence of life-threatening complications from surgery has reduced significantly. The child born with cloacal exstrophy can usually, with reconstructive surgery, achieve the ability to manage urine and stool in a socially acceptable way over time. The neurological deficit associated with spina bifida, if present, is manageable but requires ongoing medical services.

Frequently asked questions:

Will my child be able to have children when they reach adulthood?

In many cases, the answer to this question is yes. However, this will almost certainly require assisted fertility treatment.



Reviewed: January 2011

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Cloacal Exstrophy Glossary
  • abdomen: Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.

  • abdominal: in the abdomen, the cavity of this part of the body containing the stomach, intestines and bladder.

  • abnormality: A variation from a normal structure or function of the body.

  • anesthesia: Loss of sensation in any part of the body induced by a numbing or paralyzing agent. Often used during surgery to put a person to sleep.

  • anus: Opening at the end of the digestive tract where feces (stool) leave the body. The final two inches of the rectum.

  • bladder: The bladder is a thick muscular balloon-shaped pouch in which urine is stored before being discharged through the urethra.

  • bowel: Another word for intestines or colon.

  • catheter: A thin tube that is inserted through the urethra into the bladder to allow urine to drain or for performance of a procedure or test, such as insertion of a substance during a bladder X-ray.

  • clitoris: Sensitive female sex organ, which is visible at the front of the vagina.

  • colon: Large intestine.

  • continence: The ability to control the timing of urination or a bowel movement.

  • epispadias: A rare defect present at birth in which the opening of the urethra is in an abnormal location. In males, the opening is usually on the topside of the penis and not the tip.

  • exstrophy-epispadias: A congenital abnormality that affects the bladder, genitals and the pelvis.

  • fertility: The ability to conceive and have children.

  • gas: Material that results from: swallowed air, air produced from certain foods or that is created when bacteria in the colon break down waste material. Gas that is released from the rectum is called flatulence.

  • gastrointestinal: Also referred to as GI. The stomach and the intestines.

  • gastrointestinal tract: The gastrointestinal tract starts from the mouth and proceeds to the esophagus, stomach, duodenum, small intestine, large intestine, rectum and anus.

  • genitalia: External sexual organs.

  • genitourinary system: The parts of the body that play a role in reproduction and/or getting rid of waste products in the form of urine.

  • intestine: The part of the digestive system between the stomach and the anus that digests and absorbs food and water.

  • intestines: the portion of the alimentary canal extending from the stomach to the anus consisting of two segments, the small intestine and the large intestine.

  • ions: Electrically charged atoms.

  • kidney: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • neurologic: Pertaining to the nervous system.

  • neurological: Pertaining to the nervous system.

  • pelvic: Relating to, involving or located in or near the pelvis.

  • pelvis: The bowl-shaped bone that supports the spine and holds up the digestive, urinary and reproductive organs. The legs connect to the body at the pelvis.

  • penis: The male organ used for urination and sex.

  • sonogram: Also referred to as a ultrasound. A technique that bounces painless sound waves off organs to create an image of their structure to detect abnormalities.

  • spina bifida: A condition at birth in which part of the vertebral bodies (or back bones) fail to seal off completely and some part of the spinal cord protrudes through this opening. This condition is often associated with bladder and bowel control problems as well as lack of control of voluntary movement in the lower body.

  • stage: Classification of the progress of a disease.

  • stent: With regard to treating ureteral stones, a tube inserted through the urethra and bladder and into the ureter. Stents are used to aid treatment in various ways, such as preventing stone fragments from blocking the flow of urine.

  • stoma: An opening.

  • stool: Waste material (feces) discharged from the body.

  • umbilicus: Navel or belly button.

  • urge: Strong desire to urinate.

  • urinary: Relating to urine.

  • urine: Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.

  • urology: Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.

  • vagina: The tube in a woman's body that runs beside the urethra and connects the uterus (womb)to the outside of the body. Sometimes called the birth canal. Sexual intercourse, the outflow of blood during menstruation and the birth of a baby all take place through the vagina.

Cloacal Exstrophy Anatomical Drawings

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