CLOACAL EXSTROPHY

Cloacal exstrophy is a very rare and complicated birth defect. It occurs once in every 250,000 births. Cloacal exstrophy is often referred to by different labels including: Vessico Intestinal Fissure, Exstrophy of the Cloaca, Exstrophia of the Spalancia, and OEIS Complex. I find the label of OEIS Complex to be the easiest way to explain this birth defect.

OEIS Complex Description

O-Omphalocele: this is a defect of the abdominal wall, which means that the child was born with the abdominal viscera outside of the abdominal cavity, covered by a thin membrane.

E-Exstrophy of the cloaca: cloacal exstrophy consists of an open bladder and the rectum is not open in the normal location but rather communicates with the bladder.

I-Imperforate Anus: the anus has not been formed or perforated, and the colon communicates with the bladder.

S-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.

The severity of each of these anomalies will determine the plan of action for your child's life. It is best to keep in mind that although these children have similar characteristics and similar plans of action, each child's situation will be different because of the multiple defects that have occurred together or separately.

What are my child's chances of survival?

At this point in history your child has a very good chance for survival. While your child will probably not have a "normal" life, the quality of life will depend on the severity of the anomalies. Fifteen years ago, most of these children died at birth or within a few days of birth. Ten years ago, these children had a good chance to survive and thrive. Five years ago, with the progression of medical science, these children began to have futures filled with hope of being functioning, productive members of society.

Cause of this birth defect

The cause of cloacal exstrophy is unknown. It is very unlikely that you have done anything to cause this birth defect to occur. It is also unlikely that you could have done anything to prevent it from occurring. However, it can be detected prenatally.

Has my child been affected intellectually?

Most children with cloacal exstrophy are very intelligent and there is no evidence that suggests that they have a lower IQ than the rest of the population.

Is my child male or female?

If the child is born as a genetic male, most surgeons will suggest that he be changed into a female. This is done through a gonadectomy and the creation of a vagina to function normally as a female.

What types of operations will my child have?

Your child will have a series of surgeries throughout her life. Some children may have more operations than others depending on the health of the child and the doctors doing the surgery. Sometimes more than one thing may be accomplished during certain surgeries.

The first possible surgery

Soon after your child is born, the first surgery will be performed to repair the omphalocele. The surgeons will create a way for your child to eliminate waste, and will also close the bladder. Spinal defects may also be repaired at this time. At the conclusion of this surgery, your child will probably have some kind of ostomy for elimination of waste. Your child may also have an appliance to eliminate urine into.

Are these appliances permanent?

Whether or not a pull-through surgery is done to create a rectum and close the stoma depends on the amount of colon the child was born with. When the child is born with a significant amount of colon and is capable of forming solid stool, a pull-through will be performed. Even if a child cannot be continent, they can be kept completely clean with a daily enema. If the child was not born with enough colon, they will need the stoma for life. If your child has an appliance for urine, there is a possibility that this will be elininated later in life.

What next?

The next course of action will be to get your child healthy enough to go home. This will involve allowing the child to heal from his/her operations. It may also involve allowing your child to grow if he/she was born prematurely. It will involve making sure that you are capable of taking care of your child's extra needs both physically and emotionally. If you have not spoken to an Interostomal Therapist yet, do so. This is a good time to get in touch with as many support systems as possible.

What happens when we get home?

The first couple of days will be the most difficult. You will get used to it and a nurse will probably come check on you within a couple of days. Beyond the first couple of days, life should be pretty normal. The next three to four years seem to be the easiest. The birth defect that your child has is not obvious to the public. Most people will not know unless you tell them. There may be only minor problems such as susceptibility to dehydration or irritation from the appliance or urine. Usually these years will be the most problem free.

Subsequent surgeries

The major urinary reconstructive surgery is performed so that your child will be able to void urine. This is called a continent diversion, in which a neobladder will be created with a valve mechanism for intermittent catheterization. This surgery should not be performed until it is determined whether or not the child will have a permanent stoma. If a pull-through is not an option, the surgeon can use a piece of colon to enlarge the bladder. A vagina may also be created at this time.

Will my child be able to have sexual intercourse

A female will be able to have sex using her own natural genitalia. Being a male would require using a surgically made neo-vagina.

Will my child be able to have children?

In many cases, the answer to this question is also yes. This will depend on whether your child has ovaries and a uterus. If the body parts are there, it is very likely that your child will be able to have children. In fact, for some reason, these children seem to conceive very easily, as adults.

This article first appeared as "Cloacal Exstrophy" by Linda Teliha and Dr. Alberto Peña in the Pull-thru Network News, Winter 1996 and The Inside Edition November, 1996

The information provided in this section should in no way serve as medical advice. Readers are encouraged to confirm the information contained here with other sources and seek medical advice from a physician. Neither the authors nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete and they are not responsible for any errors or omissions or for the results obtained for the use of such information.

Information provided by the Division of Urology, Children's Hospital, Boston, MA.

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