16231 Prune Belly Syndrome

Overview

Prune Belly Syndrome (also called Eagle-Barrett syndrome) is a rare condition that is present from birth. It mainly affects boys and involves three main problems: weak or missing belly muscles, urinary tract issues (the system that makes and removes urine), and undescended testicles. Because the belly muscles are weak, the belly can look wrinkled, like a prune — that is where the name comes from.

No, it is rare. Most doctors will see only a few cases in their entire career, so care is usually provided by a specialist team.

It affects mainly boys, although girls can have some similar features. About 1 in 40,000 babies is born with it. Most children are diagnosed during pregnancy or in the first few months of life.

Symptoms

Common symptoms

• Weak, absent, or thin abdominal (belly) muscles, causing a wrinkled belly
• Undescended testicles in boys (testicles that have not moved down into the scrotum)
• Widened or swollen kidney tubes (dilated ureters) and a swollen kidney, called hydronephrosis
• Trouble with bladder emptying, such as a weak urine stream or needing to strain to urinate
• Frequent urinary tract infections (infections in the bladder or kidneys)

Symptoms in children

• A wrinkled, loose appearance to the belly area
• Difficulty with urination or a weak urine stream
• Repeated urinary tract infections
• Constipation because of weak belly muscles
• Breathing difficulties at birth in some babies due to underdeveloped lungs

Symptoms in older adults

• Long-term kidney problems, including decreasing kidney function
• Bladder emptying issues that may continue into adulthood
• Possible urinary leaking (incontinence) or trouble holding urine
• High blood pressure related to kidney disease

Causes

Main causes

• The exact cause is unknown. It is not caused by anything the parents did before or during pregnancy.
• Most experts think it happens early in the developing embryo, due to a problem with the tissues that form the belly wall and urinary system.
• There seems to be a genetic link in some families, but no single gene is responsible in most cases.

Risk factors

• Male sex – boys are affected much more often than girls.
• A family history of the syndrome (although this is uncommon).

When to see a doctor

See a doctor urgently if:

• If your child cannot urinate, has a fever with back pain, or has blood in the urine, seek medical care the same day.
• If an adult with Prune Belly Syndrome has sudden worsening of kidney function or severe pain, call your urology or kidney team immediately.

Book a routine appointment if:

• Routine follow-up with a pediatrician, urologist, and kidney specialist is essential, even if the child is doing well.
• Adults with the condition should have regular kidney function tests and blood pressure checks.

Diagnosis

Prune Belly Syndrome may be found during a routine pregnancy ultrasound, when a baby has a swollen belly, enlarged kidneys, or undescended testicles. After birth, a doctor can often identify it by the physical features and then confirm it with imaging tests.

Tests that may be done

• Abdominal (belly) ultrasound – uses sound waves to take pictures of the kidneys, ureters, and bladder
• Urine tests – check for infection and signs of kidney damage
• Blood tests – measure how well the kidneys are working (kidney function)
• Voiding cystourethrogram (VCUG) – an X-ray of the bladder while filling and emptying it
• Kidney scans, such as a MAG3 or DMSA scan, to see how each kidney works

What to expect at your appointment

Your healthcare team will explain the results and what they mean for your child. You will likely meet several specialists, including a kidney doctor (nephrologist), a bladder/urinary surgeon (urologist), and a physical therapist. Expect regular scans and tests over time.

Treatment

Treatment depends on how severe the urinary and kidney problems are. Some children need only monitoring and medication to keep the urinary tract healthy, while others need surgery early in life. The goal is always to protect kidney function and improve quality of life.

Self-care at home

• Help your child drink plenty of water throughout the day to keep urine moving and reduce infection risk.
• Encourage regular toileting and watch for signs of bladder emptying problems.
• Keep the belly area clean and dry, especially if the skin is irritated.
• Follow your doctor's advice on bowel care to avoid constipation, which can make bladder problems worse.

Medical treatments

There is no one medicine for Prune Belly Syndrome. Doctors may use medicines to help empty the bladder, or antibiotics to prevent urinary tract infections in some children. They may also give medicines to manage high blood pressure if the kidneys are affected. Never start or stop a medicine without speaking to the care team.

When is surgery considered?

Some babies need surgery soon after birth to help urine drain properly if a blockage is threatening the kidneys. Later, boys may need surgery to bring the testicles down into the scrotum. Some children have surgery to strengthen the belly muscles, although this is mainly done for cosmetic or functional reasons.

Living with this condition

Living with Prune Belly Syndrome means keeping a routine of medical checkups and protecting kidney health. Parents and adults should watch for signs of infection and make sure appointments with the specialist team are not missed. Physical therapy can help with belly strength and posture.

Lifestyle tips

• Stay physically active as able – walking, swimming, and cycling are often good options.
• Avoid heavy lifting or intense abdominal exercises without checking with your doctor first.
• Drink enough fluids each day to keep kidneys healthy.
• Wear comfortable clothing that does not press on the belly.
• Keep up to date with vaccinations, especially flu and pneumonia, because a chest infection can be harder to recover from.

Diet and exercise

A balanced diet with plenty of fruits, vegetables, and fibre helps prevent constipation. While there is no specific diet for Prune Belly Syndrome, eating well supports kidney function. Exercise is safe and helpful, but talk to a physiotherapist before starting anything that puts heavy strain on the belly muscles.

Mental health and emotional wellbeing

Growing up with a visible physical difference or ongoing medical procedures can be hard emotionally. Children and adults may feel anxious, frustrated, or self-conscious. It is important to talk about these feelings and get support early.

Prevention

No, Prune Belly Syndrome cannot be prevented. It occurs very early in pregnancy and is not caused by anything the parents did.

Vaccines

Vaccines are an important part of general health but do not prevent this syndrome. Keeping up with routine vaccinations helps prevent infections that can put extra strain on an already vulnerable urinary tract.

Screening programmes

Prenatal ultrasound can sometimes spot signs of Prune Belly Syndrome, but not all babies with the condition will be seen clearly on the scan. After birth, regular urine and kidney checks are the key to early detection of problems.

Complications

If left untreated

• Chronic (long-term) kidney infection and damage
• Worsening hydronephrosis, where the kidneys swell too much
• High blood pressure and eventually kidney failure
• Breathing difficulties in severe newborn cases
• Fertility issues later in life for men with undescended testicles

Long-term outlook

The outlook is better than it was in the past. With early specialist care, many people with Prune Belly Syndrome live long, active lives. Those who develop severe kidney problems may need dialysis or a kidney transplant, but many others maintain good kidney function for years. Your care team will be honest with you about what to expect, and they will help you manage challenges as they arise.

Find support

External links open third-party websites. Ruqelo Health is not responsible for external content. Listing an organisation does not imply endorsement.