Table of ContentsView AllTable of ContentsCausesSymptomsDiagnosisTreatmentOutlookSpecialists to Consult
Table of ContentsView All
View All
Table of Contents
Causes
Symptoms
Diagnosis
Treatment
Outlook
Specialists to Consult
Prune belly syndrome, also known as Eagle-Barrett syndrome or abdominal muscle deficiency syndrome, is a rare condition present at birth. It affects about 1 in every 30,000 to 40,000 children. The name describes the way the skin on children’s abdomens (bellies) wrinkles, resembling a prune.
Prune belly syndrome is also known as triad syndrome because three anatomical differences define it, as follows:
Prune belly syndrome affects everyone differently. It can involve mild to severe problems with the bladder,ureter(the tube that connects the bladder and kidneys), andkidneys. Other anatomical variations in the skeletal system, intestines, and heart, among other health problems, can also occur. Treatment can range from medication to surgery, based on the severity of the anomalies.
This article describes prune belly syndrome, its causes, symptoms, and diagnosis. It also explains the treatments and outlook for this problem.
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What Causes Prune Belly Syndrome?
The exact cause of prune belly syndrome is unknown. Research indicates that the condition iscongenital(present from birth) with no known environmental causes. In 95% of babies affected with prune belly syndrome, the sex assigned at birth is male.
Prune belly syndrome develops as the fetus grows before birth. One theory is that the condition results from a blockage in the urethra (the tube that drains urine from the bladder to the outside of the body). If a blockage exists, urine builds up in the bladder, causing pressure and swelling.
When the fluid drains after birth, it leaves a wrinkled abdomen that resembles a prune. The shriveled skin is more noticeable since there is also a lack of healthy abdominal muscles.
Some cases of prune belly syndrome have occurred in siblings. While this indicates a possible genetic factor to the disease, the link is not well understood.
The majority of prune belly syndrome cases remain unexplained.Further research is needed to utilize this knowledge in genetic counseling and early detection.
Prune Belly Syndrome Symptoms
Prune Belly Syndrome Diagnosis
Prune belly syndrome is usually obvious from birth, but it takes time to determine the location and extent of abnormalities. A physical exam shows the belly wall’s obvious wrinkled appearance and undescended testicles in babies assigned male at birth.
In addition to the physical exam, a prune belly diagnosis is made with a combination of the following tests:
Prune Belly Syndrome Treatment
Every case of prune belly syndrome is unique, so treatment depends on a person’s symptoms, the severity of their condition, age, and general health. Goals for prune belly syndrome treatment include preserving kidney function and preventing urinary tract infections.
Surgery of the abdominal wall and urinary tract reconstruction are often required. When possible, surgeries are combined to reduce the number of operating room trips. These procedures often include:
People with prune belly disease are typically born with poorly formed or dilated kidneys. They produce a lot of urine, which is dilute (mostly water, less concentrated than typical urine).
Most people with prune belly syndrome are unable to fully empty their bladders because they lack strong abdominal muscles. This makes them likely to have frequent urinary tract infections. Children may be placed on prophylactic (preventive) antibiotics to help prevent UTIs.
Treating prune belly syndrome typically requires a large multidisciplinary approach involving various medical specialists. Some specialists that may provide treatment and support for associated conditions include:
What’s the Outlook for Someone With Prune Belly Syndrome?
There is no cure for prune belly syndrome. The outlook for children and adults with this condition varies significantly based on disease severity and the defects involved. Many people have good physical and mental health through adulthood.
Individualized care and improvements in the evaluation, management, and surgical treatment of common defects of prune belly syndrome have improved the overall outlook for survival and quality of life for those affected. Prenatal diagnosis and pregnancy termination have contributed to a declining incidence of prune belly syndrome in developed countries.
While some newborns with prune belly syndrome survive and continue to normal development, others have numerous medical and developmental problems throughout their lives. In some cases, the results are fatal. Severe lung or kidney problems and other severe anatomical differences can cause stillbirth or death within the first few weeks of life.
A significant factor in long-term survival involves the severity of the urinary tract involvement, especially the failure of the kidney to fully develop in the fetus (kidney dysplasia).
What to Do If You Suspect Prune Belly Syndrome in Your Child
The diagnosis of prune belly syndrome is typically made before or at birth. The condition causes physical changes that are hard to overlook. However, some less-severe cases or cases that do not have the typical outward signs of prune belly syndrome may not be noticeable until later in a child’s life, resulting in a delayed diagnosis.
A child with prune belly syndrome is likely to get frequent UTIs. Monitor how frequently your child urinates. Signs of a UTI in children include:
If you suspect that your child has a UTI or other signs of prune belly syndrome, contact your healthcare provider immediately. When newborns are diagnosed, treatments and surgical intervention begin as early as possible to reduce the risk of kidney failure and other complications.
Summary
Prune belly syndrome is a rare condition present at birth that involves problems that range from mild to severe. Babies born with this ailment have three main anatomical differences. These problems include weak or missing abdominal muscles, one or both undescended testicles, and an abnormally large bladder with kidney problems.
Symptoms vary since prune belly syndrome affects each person uniquely. It is common to have frequent UTIs, which can lead to kidney damage. Other anatomical anomalies can impact the intestines, lungs, and heart.
Prune belly syndrome is most often found in utero or at birth. Early diagnosis can help babies get the right treatment as soon as possible for the best results.
The effects of prune belly syndrome are severe or deadly for some babies. However, many babies have minor defects and remain in good physical and mental health through adulthood. No matter the disease severity, anyone with prune belly syndrome needs lifelong follow-up and assessment due to gradual kidney damage and other health problems that often occur.
Bladder Exstrophy: From Diagnosis to Repair
9 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Organization for Rare Disorders.Prune belly syndrome.Prune Belly Syndrome Network.Building a better world for children.Orange County Children’s Hospital.Prune belly syndrome.Lopes RI, Baker LA, Dénes FT.Modern management of and update on prune belly syndrome.J Pediatr Urol. 2021;17(4):548-554. doi:10.1016/j.jpurol.2021.04.010Children’s Hospital of Philadelphia (CHOP).Prune belly syndrome.Mount Sinai.Prune belly syndrome.University of California, San Francisco.Prune belly syndrome.Nationwide Children’s Hospital.Prune belly syndrome.Dénes FT, Caldamone AA. (2022).Prune-belly syndrome. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. doi:10.1007/978-3-642-38482-0_186-1
9 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Organization for Rare Disorders.Prune belly syndrome.Prune Belly Syndrome Network.Building a better world for children.Orange County Children’s Hospital.Prune belly syndrome.Lopes RI, Baker LA, Dénes FT.Modern management of and update on prune belly syndrome.J Pediatr Urol. 2021;17(4):548-554. doi:10.1016/j.jpurol.2021.04.010Children’s Hospital of Philadelphia (CHOP).Prune belly syndrome.Mount Sinai.Prune belly syndrome.University of California, San Francisco.Prune belly syndrome.Nationwide Children’s Hospital.Prune belly syndrome.Dénes FT, Caldamone AA. (2022).Prune-belly syndrome. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. doi:10.1007/978-3-642-38482-0_186-1
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Organization for Rare Disorders.Prune belly syndrome.Prune Belly Syndrome Network.Building a better world for children.Orange County Children’s Hospital.Prune belly syndrome.Lopes RI, Baker LA, Dénes FT.Modern management of and update on prune belly syndrome.J Pediatr Urol. 2021;17(4):548-554. doi:10.1016/j.jpurol.2021.04.010Children’s Hospital of Philadelphia (CHOP).Prune belly syndrome.Mount Sinai.Prune belly syndrome.University of California, San Francisco.Prune belly syndrome.Nationwide Children’s Hospital.Prune belly syndrome.Dénes FT, Caldamone AA. (2022).Prune-belly syndrome. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. doi:10.1007/978-3-642-38482-0_186-1
National Organization for Rare Disorders.Prune belly syndrome.
Prune Belly Syndrome Network.Building a better world for children.
Orange County Children’s Hospital.Prune belly syndrome.
Lopes RI, Baker LA, Dénes FT.Modern management of and update on prune belly syndrome.J Pediatr Urol. 2021;17(4):548-554. doi:10.1016/j.jpurol.2021.04.010
Children’s Hospital of Philadelphia (CHOP).Prune belly syndrome.
Mount Sinai.Prune belly syndrome.
University of California, San Francisco.Prune belly syndrome.
Nationwide Children’s Hospital.Prune belly syndrome.
Dénes FT, Caldamone AA. (2022).Prune-belly syndrome. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. doi:10.1007/978-3-642-38482-0_186-1
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