Learn About Prune Belly Syndrome

What is the definition of Prune Belly Syndrome?

Prune belly syndrome is a group of rare birth defects that involves these three main problems:

  • Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune
  • Undescended testicles
  • Urinary tract problems
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What are the alternative names for Prune Belly Syndrome?

Eagle-Barrett syndrome; Triad syndrome

What are the causes of Prune Belly Syndrome?

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

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

What are the symptoms of Prune Belly Syndrome?

Weak abdominal muscles can cause:

  • Constipation
  • Delay in sitting and walking
  • Difficulties coughing

Urinary tract problems can cause difficulty urinating.

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What are the current treatments for Prune Belly Syndrome?

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.

Who are the top Prune Belly Syndrome Local Doctors?
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Universidade De São Paulo

Sao Paulo, SP, BR 

Francisco Denes is in Sao Paulo, Brazil. Denes is rated as an Elite expert by MediFind in the treatment of Prune Belly Syndrome. He is also highly rated in 5 other conditions, according to our data. His top areas of expertise are Prune Belly Syndrome, UPJ Obstruction, Abdominal Wall Surgery, and Obstructive Uropathy.

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Highly rated in
11
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Universidade De São Paulo

Sao Paulo, SP, BR 

Roberto Lopes is in Sao Paulo, Brazil. Lopes is rated as an Elite expert by MediFind in the treatment of Prune Belly Syndrome. He is also highly rated in 11 other conditions, according to our data. His top areas of expertise are Prune Belly Syndrome, Ureterocele, UPJ Obstruction, and Hydronephrosis.

 
 
 
 
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Elite
Highly rated in
12
conditions

Hospital Federal Da Lagoa

Rio De Janeiro, RJ, BR 

Luciano Favorito is in Rio De Janeiro, Brazil. Favorito is rated as an Elite expert by MediFind in the treatment of Prune Belly Syndrome. He is also highly rated in 12 other conditions, according to our data. His top areas of expertise are Prune Belly Syndrome, Undescended Testicle, Urethral Stricture, and Anencephaly.

What are the support groups for Prune Belly Syndrome?

More information and support for people with prune belly syndrome and their families can be found at:

  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/prune-belly-syndrome
  • Prune Belly Syndrome Network -- prunebelly.org
What is the outlook (prognosis) for Prune Belly Syndrome?

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

Many infants with this condition are either stillborn or die within the first few weeks of life. The cause of death is from severe lung or kidney problems, or from a combination of birth problems.

Some newborns survive and can develop normally. Others continue to have many medical and developmental problems.

What are the possible complications of Prune Belly Syndrome?

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

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

Undescended testicles can lead to infertility or cancer.

When should I contact a medical professional for Prune Belly Syndrome?

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

If you have a child with diagnosed prune belly syndrome, contact 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 swollen bladder or enlarged kidneys, talk to a specialist in high-risk pregnancy or perinatology.

How do I prevent Prune Belly Syndrome?

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.

What are the latest Prune Belly Syndrome Clinical Trials?
Validation of the Standardized Prenatal Clinical Management of Fetuses With Lower Urinary Tract Obstruction (LUTO)
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What are the Latest Advances for Prune Belly Syndrome?
Long-term Outcome of Renal Transplantation in Patients with Congenital Lower Urinary Tract Malformations: A Multicenter Study.
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Who are the sources who wrote this article ?

Published Date: November 01, 2021
Published By: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Denes FT, Lopes RI. Prune-belly syndrome. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021:chap 32.

Elder JS. Obstruction of the urinary tract. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 555.

Merguerian PA, Rowe CK. Developmental abnormalities of the genitourinary system. In: Gleason CA, Juul SE, eds. Avery's Diseases of the Newborn. 10th ed. Philadelphia, PA: Elsevier; 2018:chap 88.