Prune Belly Syndrome
Access exceptional care for prune belly syndrome at Montefiore Einstein Urology at the Children’s Hospital at Montefiore Einstein (CHAM). As a leader in urology, we are an academic-based referral site for the most complex and high-risk cases. For more than a century, we have been at the forefront of innovation, research and clinical care for genitourinary (GU) conditions.
Montefiore Einstein continues to make advances in the diagnosis and treatment of GU conditions. Our multidisciplinary team of specialists is passionate about uncovering diagnostic approaches and treatments that can improve outcomes.
At Montefiore Einstein Urology, you can expect compassionate research-based treatment plans that meet the highest standards for quality and safety. We are committed to ensuring your child will receive the best patient- and family-centered care in a supportive and nurturing environment.
When you want only the best for your child, turn to our dedicated providers who will develop a highly personalized treatment plan.
What Is Prune Belly Syndrome
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare congenital condition characterized by the partial or complete absence of abdominal muscles, urinary tract abnormalities and the failure of both testicles to descend into the scrotum during fetal development. Complications associated with this condition include underdevelopment of the lungs, renal disorders or failure, and skeletal or heart abnormalities. This disorder affects around 1 in 40,000 newborns, primarily males.
Causes of Prune Belly Syndrome
The exact cause of prune belly syndrome is unknown. Some researchers theorize that the condition may be associated with abnormal fetal development of the bladder or incomplete formation of the abdominal muscles, while others believe that there is an undiscovered common cause of abnormalities in both the bladder and abdomen.
Signs & Symptoms of Prune Belly Syndrome
Symptoms of prune belly syndrome vary widely according to the severity of the condition. Common symptoms include:
- A wrinkled, prune-like appearance of the stomach
- Undescended testicles
- Abdominal bulge/distention with a thin wall through which the intestinal loops can be seen
- Urinary tract conditions, including enlarged bladder, enlarged or blocked ureters and urine reflux into the kidney
- Gastrointestinal and intestinal conditions
- Musculoskeletal conditions
- In severe cases, lung and cardiovascular abnormalities
Diagnosing Prune Belly Syndrome
Often prune belly syndrome is diagnosed during routine fetal ultrasounds. If not diagnosed prenatally, it is typically quickly diagnosed based on the distinctive appearance of the abdomen. Additional testing is required to determine the extent of the condition and which organs may be affected. To reach a comprehensive diagnosis, your child’s physician may order imaging tests, including X-rays, ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT) scans.
Treating Prune Belly Syndrome
Treatment for prune belly syndrome depends on the number of organs affected and the severity of the overall condition. Surgery may be recommended to rebuild the urinary tract and abdominal wall, while male patients require surgery to lower the testicles into the scrotum. In all cases, preserving kidney and urinary function remains a high treatment priority. In rare cases, more extensive surgeries are required to repair abnormalities and restore full functionality.