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    Prune belly syndrome

    Eagle-Barrett syndrome; Triad syndrome; Urethral obstruction malformation sequence

    Prune belly syndrome is a group of birth defects that involve three main problems:

    • Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune
    • Undescended testicles (cryptorchidism)
    • Urinary tract problems

    Causes

    The causes of prune belly syndrome are unknown. The condition affects mostly boys.

    While in the womb, the developing baby's abdomen swells with fluid. That fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. The appearance is more noticeable due to the lack of abdominal muscles.

    Symptoms

    Weak abdominal muscles can cause:

    • "Little Buddha" appearance
    • Constipation
    • Delay in sitting and walking
    • Difficulties coughing

    Urinary tract problems can cause difficulty urinating.

    Exams and Tests

    A woman who is pregnant with a baby who has prune belly syndrome may not have enough amniotic fluid (oligohydramnios). This can cause the infant to have lung problems.

    An ultrasound done during pregnancy may show that the baby has a swollen bladder or enlarged kidney.

    In some cases, a pregnancy ultrasound may also help determine if the baby has:

    • Heart problems
    • Bone/muscle (musculoskeletal) abnormalities
    • Stomach and intestinal problems
    • Underdeveloped lungs

    The following tests may be performed on the baby after birth to diagnose the condition:

    • Blood tests
    • Intravenous pyelogram (IVP)
    • Ultrasound
    • Voiding cystourethrogram (VCUG)
    • X-ray

    Treatment

    Early surgery is recommended to fix weak abdominal muscles, urinary tract problems, and undescended testicles.

    The baby may be given antibiotics to treat or help prevent urinary tract infections.

    Outlook (Prognosis)

    Prune belly syndrome is a serious and often life-threatening problem.

    Many infants with prune belly syndrome are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems.

    Some newborns survive but continue to have problems.

    Possible Complications

    Complications depend on the related problems. The most common are:

    • Constipation
    • Bone deformities (clubfoot, dislocated hip, missing limb or digit, funnel chest)
    • Disease of the urinary tract (patients may need dialysis and a kidney transplant)

    Undescended testicles can lead to infertility or cancer.

    When to Contact a Medical Professional

    Prune belly syndrome is usually diagnosed before birth or when the baby is born.

    If you have a child with diagnosed prune belly syndrome, call your health care provider at the first sign of a urinary tract infection or other urinary symptoms.

    If a pregnancy ultrasound shows that your baby has a distended bladder or enlarged kidneys, talk to a specialist in high-risk pregnancy or perinatology.

    Prevention

    There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.

    References

    Caldamone AA, Woodard JR. Prune belly syndrome. In: Wein AJ, ed. Campbell-Walsh Urology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 118.

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                Review Date: 11/14/2011

                Reviewed By: Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review Provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

                The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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