Overview
Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome or triad syndrome, is a rare congenital condition characterized by three cardinal features: deficiency or absence of the abdominal wall musculature (giving the abdomen a wrinkled, prune-like appearance), bilateral cryptorchidism (undescended testes) in males, and urinary tract abnormalities including megacystis (enlarged bladder), dilated ureters (megaureters), and hydronephrosis. The condition predominantly affects males, accounting for approximately 95% of cases. The urinary tract abnormalities are the most clinically significant aspect of the syndrome, as they can lead to recurrent urinary tract infections, vesicoureteral reflux, and progressive renal impairment that may ultimately result in end-stage kidney disease. The deficient abdominal musculature can cause respiratory complications due to impaired cough mechanics and difficulty with pulmonary clearance. Additional associated anomalies may include cardiac defects, gastrointestinal malformations (such as intestinal malrotation), and musculoskeletal abnormalities including hip dysplasia and clubfoot. The severity of the condition varies widely, ranging from a lethal form with renal dysplasia and pulmonary hypoplasia (often associated with oligohydramnios) to milder presentations compatible with a relatively normal life. Treatment is multidisciplinary and depends on the severity of the condition. Management of urinary tract abnormalities may include prophylactic antibiotics to prevent infections, intermittent catheterization, and surgical reconstruction of the urinary tract (such as ureteral reimplantation or vesicostomy). Orchiopexy is performed to bring undescended testes into the scrotum. Abdominoplasty may be considered to improve abdominal wall function and cosmetic appearance. In severe cases with renal failure, dialysis or kidney transplantation may be necessary. Early diagnosis, often possible prenatally through ultrasound, allows for coordinated postnatal care planning.
Also known as:
Clinical phenotype terms— hover any for plain English:
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
1 eventMaria Sklodowska-Curie National Research Institute of Oncology
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Prune belly syndrome.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Prune belly syndrome.
Community
No community posts yet. Be the first to share your experience with Prune belly syndrome.
Start the conversation →Latest news about Prune belly syndrome
No recent news articles for Prune belly syndrome.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Common questions about Prune belly syndrome
What is Prune belly syndrome?
Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome or triad syndrome, is a rare congenital condition characterized by three cardinal features: deficiency or absence of the abdominal wall musculature (giving the abdomen a wrinkled, prune-like appearance), bilateral cryptorchidism (undescended testes) in males, and urinary tract abnormalities including megacystis (enlarged bladder), dilated ureters (megaureters), and hydronephrosis. The condition predominantly affects males, accounting for approximately 95% of cases. The urinary tract abnormalities are the most clinically signific
At what age does Prune belly syndrome typically begin?
Typical onset of Prune belly syndrome is neonatal. Age of onset can vary across affected individuals.
Are there clinical trials for Prune belly syndrome?
Yes — 1 recruiting clinical trial is currently listed for Prune belly syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Prune belly syndrome?
11 specialists and care centers treating Prune belly syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.