Skip to content

We are currently in beta

Please enjoy the existing content while we continue to fix and enhance your experience.

We would love to receive any feedback or comments that you'd like to share.

You can contact us here

Journeys of Life StoryMD Health Communities

Why StoryMD
Our Story
Point of Care
Newsletter
Contact Us
Help Center

Privacy Policy
Terms of Service
Accessibility

Find us on facebook
Find us on instagram
Find us on linkedin
Find us on pinterest
Find us on twitter
Find us on youtube

© 2024 StoryMD Inc. All rights reserved.

Prune Belly Syndrome

Table of Contents

Prune Belly Syndrome

PBS; Eagle-Barrett Syndrome; Obrinsky Syndrome; Triad Syndrome

Prune belly syndrome, or PBS, is a group of birth defects characterized by a lack of abdominal muscles, causing the skin on the abdominal area to wrinkle and appear "prune-like"; undescended testicles in males; and urinary tract malformations. Explore symptoms, causes, and genetics.

Baby

Image by predvopredvo/Pixabay

Summary

Prune belly syndrome - A. At birth B. At nine years of age (surgical scars noted from a prior abdominoplasty procedure)

Image by Senthilkumar Sankararaman, MD - Copyright © 2023, StatPearls Publishing LLC

Prune belly syndrome - A. At birth B. At nine years of age (surgical scars noted from a prior abdominoplasty procedure)

Image by Senthilkumar Sankararaman, MD - Copyright © 2023, StatPearls Publishing LLC

What Is Prune Belly Syndrome?

Prune belly syndrome (PBS) is a condition characterized by a lack of abdominal muscles, causing the skin on the abdominal area to wrinkle and appear "prune-like"; undescended testicles in males; and urinary tract malformations. PBS is more common in males. The severity of symptoms can vary greatly from person to person. At one end of the spectrum, the condition may cause severe kidney and pulmonary (lung) problems incompatible with life (resulting in stillbirth); at the other end of the spectrum, the condition may cause few, if any, urological abnormalities. The cause of PBS is unknown. Treatment varies, but usually includes surgical management of symptoms.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (2)

Prune Belly Syndrome PSA

Video by Prune Belly Syndrome/YouTube

2-Year-Old With Prune Belly Syndrome Gets Kidney Transplant

Video by Inside Edition/YouTube

1:14

Prune Belly Syndrome PSA

Prune Belly Syndrome/YouTube

1:04

2-Year-Old With Prune Belly Syndrome Gets Kidney Transplant

Inside Edition/YouTube

Causes

Unknown (película)

Image by Diseñadores publicitarios/Wikimedia

Unknown (película)

Logo de «Unknown».

Image by Diseñadores publicitarios/Wikimedia

What Causes Prune Belly Syndrome?

CHRM3 gene has been reported in one family with a history of prune belly syndrome. Otherwise, an underlying genetic cause has not been identified.

There are several theories regarding the development of prune belly syndrome. Some researchers think it may occur if there is a blockage preventing the flow of urine through the urinary tract. The blockage can cause the urine to flow back into the bladder, enlarging it. While other researchers consider the urinary abnormalities to be secondary to the incomplete development of the bladder.

Source: Genetic and Rare Diseases (GARD) Information Center

Symptoms

Prune belly syndrome

Image by Kotb A Metwalley, Hekma S Farghalley and Alaa A Abd-Elsayed

Prune belly syndrome

Prune belly syndrome in an Egyptian child with Down syndrome.

Image by Kotb A Metwalley, Hekma S Farghalley and Alaa A Abd-Elsayed

What Are the Signs and Symptoms of Prune Belly Syndrome?

Common symptoms include:

Poorly developed and/or absent abdominal muscles
Undescended testicles in males (cryptorchidism)
Urinary tract problems such enlarged or blocked ureters (tube that carries urine from the bladder outside the body)
Enlarged bladder
Enlarged kidney (hydronephrosis)

Other symptoms might include:

Cardiac defects
Spine malformations
Club foot
Gastrointestinal anomalies

Source: Genetic and Rare Diseases (GARD) Information Center

Treatment

Surgery

Image by Staff Sgt. Miguel Lara/Wikimedia

Surgery

U.S. Air Force Maj Jonathan Forbes, 455th Expeditionary Medical Operations Squadron neurosurgeon looks through loupes as he performs brain surgery at the Craig Joint Theater Hospital, Bagram Air Field, Afghanistan, Oct. 10, 2014. The loupes allow Forbes to see the surgery at a two times magnification. Forbes removed an 8-centimeter tumor during the five-hour surgery. Forbes is deployed from Travis Air Force Base, Calif., and is a native of New Martinsville, W.Va. (U.S. Air Force photo by Staff Sgt. Miguel Lara III/Released) Unit: 455th Air Expeditionary Wing

Image by Staff Sgt. Miguel Lara/Wikimedia

How Might Prune Belly Syndrome Be Treated?

In general, surgery may be done to repair abdominal muscle, genital, and bladder problems. Antibiotics may be given to infants to treat or prevent urinary tract infections. Timing of therapy may vary from patient to patient.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (1)

2-Year-Old With Prune Belly Syndrome Gets Kidney Transplant

Video by Inside Edition/YouTube

1:04

2-Year-Old With Prune Belly Syndrome Gets Kidney Transplant

Inside Edition/YouTube

Prognosis

Treatment and Prognosis varies depending on the type of condition and the age of symptom onset.

Image by 4144132

Treatment and Prognosis varies depending on the type of condition and the age of symptom onset.

Prognosis varies depending on the type of MND and the age of symptom onset.

Image by 4144132

What Is the Long-Term Outlook for People with Prune Belly Syndrome?

The prognosis associated with prune belly syndrome varies depending several factors including the severity of the underlying tract anomaly, how well the kidneys are developed, and the likelihood of renal failure. The condition can become life threatening in severely affected children; however, mild cases might be limited to undescended testicles and a small amount of abdominal wall laxity. Studies have found that 30% of individuals with prune belly syndrome require kidney transplantation in their lifetime.

Despite these concerns, many individuals with prune belly syndrome report having good physical and mental health as well as a good overall quality of life.

Source: Genetic and Rare Diseases (GARD) Information Center

Related HealthJournals

Infant Health

Some physical conditions and issues are very common during the first couple of weeks after birth. Many are normal, and the infant's caregivers can deal with them if they occur. Better understand some common infant health issues new parents and caregivers may need to contend with.

Baby Health Checkup

Baby health checkups are important in making sure that your baby is growing and developing properly. If there are problems, you can catch them early. Understand the importance of regular checkups for your baby.

Newborn Screening

A newborn infant has screening tests that must be done during the first few days of life. These tests look for serious medical conditions that can cause lifelong health problems or early death. With early diagnosis, treatment can begin right away, before serious problems can occur or become permanent. Get the facts about these tests.

Share and discuss

Share your story, discuss an issue or get
advice from the community

Create conversation

Sign up here for full access to StoryMD

Get free access to in-depth articles and track your personal health.

Create Free Account Sign In

Share with others

Send this HealthJournal to your friends or across your social medias.

Prune Belly Syndrome

Prune belly syndrome, or PBS, is a group of birth defects characterized by a lack of abdominal muscles, causing the skin on the abdominal area to wrinkle and appear "prune-like"; undescended testicles in males; and urinary tract malformations. Explore symptoms, causes, and genetics.

Copy Link