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Cloacal Deformities

  • Cloacal deformities encompass a wide range of complicated defects in girls that occur during fetal development of the lower abdominal structures, particularly the intestinal and genito-urinary tracts, raising concerns about urinary control, bowel control and eventual sexual function. There are many variations of these congenital defects.

    How does Boston Children’s Hospital treat cloacal deformities?

    Cloacal deformities require surgical repair. Children’s physicians and surgeons have extensive experience in repairing a wide range of cloacal deformities. We can help, no matter what condition your child has — and can start planning interventions even before your child is born.

    Contact Us

    Urology

    Boston Children's Hospital
    300 Longwood Avenue
    Boston, MA  02115


     617-355-7796



  • What kinds of cloacal deformities are there?

    There are many variations of cloacal deformities, but most involve the opening of the urethra (the tube through which urine leaves the body), the genital tract and the rectum. Normally, each of these channels has a separate opening at the skin’s surface, but in this condition, the three tracts merge into one common channel and one opening. The ovaries are typically normal. 

    Specific defects may include:

    • variation in the degree of development of the bladder, reproductive organs, abdominal wall and rectum

    • vaginal duplication or atresia (absence or abnormal closure of the vagina)

    • lack of or failure in the development of other female reproductive structures such as the uterus and fallopian tubes

    • imperforate anus, a condition where the anus has not been formed or is perforated, and the colon connects to the bladder

    Cloacal deformities are often associated with the following conditions:

    • anomalies of the upper urinary system, sometimes including anomalies in one or both kidneys
    • anomalies of the bones in the pelvis or lower spine
    • anomalies of the spinal cord and, sometimes, the affected nerves

    Cloacal exstrophy

    The most complicated cloacal deformity is cloacal exstrophy, a severe birth defect in which a child is born with many intra-abdominal structures, such as the large intestine and bladder, exposed. An omphalocele (a birth defect in which abdominal organs stick out of the navel) will often be present as well.   Please see more detailed information in our page on Cloacal Exstrophy.

    What causes cloacal deformities?

    The cause of cloacal deformities is unknown. It does not appear that any medications or activities of the parents or expectant mother have any impact on the development of cloacal deformities. Based on our current knowledge, it is very unlikely that this condition can be prevented.

  • How will my physician diagnose a cloacal deformity in my child?

    In some instances, cloacal deformities can be diagnosed before birth with a prenatal ultrasound and may be confirmed by magnetic resonance imaging (MRI). After birth, these diagnoses may be confirmed or determined by physical examination.

    Your physician will likely conduct a careful analysis of structures of the upper urinary system and sacrum, as well as perineal and pelvic anatomy, using some combination of the following diagnostic imaging studies:

    • computerized tomography scan (also called a CT or CAT scan)

    • magnetic resonance imaging (MRI)

    • endoscopy - insertion of a small instrument called an endoscope to view the interior of a hollow organ, such as the rectum, urethra or vagina

    • abdominal ultrasound (also called sonography) - an imaging method used to view the anatomy of the internal organs as they function, and to assess blood flow

    Your child may also be referred to undergo evaluation for esophageal, vertebral, tracheal, radial or cardiac anomalies that are sometimes associated with cloacal deformities.

  • How are cloacal deformities treated?

    Cloacal deformities require surgical repair. The treatment plan devised for your child will depend on the type and extent of the abnormality.

    Stabilizing your newborn

    Before a treatment plan to correct your baby's anatomy is put in place, your doctors' immediate concern will be to stabilize her condition. This may involve the creation of a diverting colostomy to allow the passage of stool. With a colostomy, the large intestine is divided into two sections and the ends of the intestine are brought through surgically created openings (stomas) in the abdomen. The upper section allows stool to pass into a collection bag, while the lower section allows for drainage of mucus produced by the intestine.

    The urinary bladder may also need to be decompressed to relieve obstruction of urine flow from the bladder and, at times, the kidney. Some children will be able to void urine on their own, but for others, intermittent catheterization may be needed to help eliminate urine. The vagina must also be decompressed to allow it to drain.

    Surgical correction

    After stabilization, and when your baby has had a chance to grow, the anatomic features of the deformity will be clarified, and a definitive correction will be planned. The treatment typically involves the surgical creation of a urethra and vagina. In some children with less severe deformity, the urethra and vagina are intact and they do not need to be created, but simply have their openings brought to the skin surface. Finally, a reconstruction of the bowel, through a procedure called a "pull-through" of the colon, must be completed. For this procedure, the surgeon may have to open the abdomen to complete the connection of the colon to the rectum.

    Follow-up and further surgery

    Once healing is assured, a third operation will close the colostomy and reestablish normal bowel movements through the rectum. Further urinary or genital tract surgery may also be needed.

    What is the long-term outlook for a child with a cloacal deformity?

    The outlook for a child with a cloacal deformity depends on a number of factors, including the extent of the problem, associated anomalies, whether the sacrum is affected, and the technical exactness and healing of the operative procedure used to correct the problem. Physicians and surgeons at Children's have a great deal of experience treating children with all kinds of cloacal deformities.

    • Mild deformity - More mild cases generally have a favorable long-term outlook. These children usually have excellent bladder control, normal sexual development and function (including fertility) and a near normal pattern of bowel movements.

    • Severe deformity - 70 to 80 percent of children will have a very favorable outlook with good to excellent stool control and only infrequent episodes of leakage or incontinence. The other 20 to 30 percent, depending on the degree of associated problems, will likely need a life-long bowel and/or urinary bladder management program with a variety of medical and surgical interventions (as needed) to improve their quality of life.

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