Prune Belly Syndrome

Characteristics of Prune Belly Syndrome

Prune-belly syndrome is characterized by three main problems:

• Abnormal or severely weakened abdominal muscles.
• One or both testicles are undescended in male children.
• Enlarged bladder and problems with the kidneys and ureters (the tubes that carry urine from the kidneys to the bladder). A child with prune belly syndrome may not be able to fully empty their bladder.

Children with prune-belly syndrome sometimes have other congenital birth defects that affect the heart, intestines, lungs, and skeletal system.

Many children with very severe prune-belly syndrome will not survive, resulting in miscarriage, stillbirth or dying in the first few months of life. Others could end up with kidney failure, and later on require a kidney transplant. However, mild to moderate forms of the condition can be managed with surgery and other treatments, allowing children to live full and active lives.

What Causes Prune Belly Syndrome?

Experts don’t know what causes prune belly syndrome, but believe there may be a genetic component. One of the theories is that it may occur if there is a blockage of the tube that carries urine out of the body from the bladder (urethra) during fetal development. This can cause urine to flow back up to the bladder, causing it to become enlarged.

Prune-belly syndrome is a rare disorder that occurs in about 1 in 30,000 to 40,000 births. The condition is more common in boys.

Prune Belly Syndrome Symptoms

Symptoms of prune belly syndrome can range from mild to severe, depending on the child. Some of the most common include:

• Belly that looks wrinkly, resembling a prune.
• Bladder and other urinary tract organs can be felt or seen through the belly.
• Urinary tract issues, including:
  • Dilated or twisted ureters, which can cause a megaureter and/or vesicoureteral reflux.
  • Enlarged or poorly developed kidneys.
  • Urinary tract infections (UTIs).
  • Underdeveloped urethra.
• Undescended testicles in boys.
• Respiratory issues due to abnormal abdominal muscles
• Heart structural problem.
• Outline of the intestines seen through the belly.
• Trouble sitting upright or walking because of weak muscles in the belly.

Potential Complications

The most common complications of prune belly syndrome include:

• Bone deformities
• Chronic kidney failure
• Constipation
• Infertility or cancer due to undescended testicles
• Pulmonary hyperplasia (underdeveloped lungs)
• Urinary tract diseases

Diagnosing Prune Belly Syndrome

Prune belly syndrome is often diagnosed by ultrasound before a child is born or shortly after birth during the newborn’s first physical exam. When prune belly is diagnosed in older children, it’s often because the child is experiencing frequent UTIs. The child’s healthcare provider will perform a physical exam and discuss the child’s symptoms and medical history. Other diagnostic tests may include:

• Blood tests: To assess kidney function.
• Kidney ultrasound: This imaging test uses sound waves to create images of the kidneys.
• Voiding cystourethrogram (VCUG): A catheter is placed through your child’s urethra into the bladder, slowly filling the bladder with a contrast solution. A special machine called fluoroscopy takes pictures. The test can determine if any of the contrast is going back up the ureters toward the kidneys, which indicates the presence and degree of urine reflux.
• CT scan: An imaging test that puts X-ray images together to create pictures of abdomen and pelvis anatomy.
• Dinamic Cintagram (MAG3): This test uses an X-ray and contrast dye to see the urinary tract. It can show the rate and path of urine flow within the urinary tract and monitor kidney function.

Prune Belly Treatment

Treatment will depend on your child’s symptoms, age, general health and the severity of the condition. Treatment options include:

Medication: Children with mild prune belly syndrome may be prescribed antibiotics to prevent UTIs.

Surgery: Some children may require one or more surgeries to repair problems with the urinary tract and other organs. Procedures include:

• Vesicostomy: This surgery makes a small opening in the belly through which the bladder can be emptied.
• Orchiopexy: A surgery done in boys to bring the undescended testicles into the scrotum.
• Cystoplasty: This advanced procedure involves a complete bladder reconstruction.
• Abdominoplasty: to provide support to the abdominal cavity, and facility the pulmonary development.

Talk with your child’s healthcare provider about the benefits and risks of these treatments. Your child will most likely require follow-up care with their pediatric urologist and other specialists as needed to address any health problems or complications related to prune belly syndrome and its treatments.