Treatment Strategies: Reconstructive Surgery, Kidney Care, and Daily Life

Treatment for Prune Belly Syndrome (PBS) is highly individualized. Your medical team will create a customized plan focusing on protecting kidney function, improving abdominal strength, ensuring healthy reproductive development, and supporting daily physical growth. ^[1][2]

Urological and Kidney Care

The most urgent, ongoing priority is ensuring urine flows freely out of the body so it does not back up and damage the kidneys. ^[1]

• UTI Prevention: Because children with PBS have large, floppy bladders and ureters that do not empty completely, urine can pool (urinary stasis). This puts them at an extremely high risk for Urinary Tract Infections (UTIs), which can quickly lead to severe kidney infections or sepsis. ^[3] Doctors often prescribe daily prophylactic (preventative) antibiotics and require parents to seek immediate medical care for any unexplained fever. ^[4]
• Clean Intermittent Catheterization (CIC): If the bladder cannot squeeze well enough to empty fully, parents (and eventually the children themselves) must learn to perform CIC. This involves inserting a small, clean tube through the urethra into the bladder several times a day to drain the urine. This is a crucial, routine part of daily life for many with PBS. ^[1]
• Vesicostomy and Surgery: If the child is too small for CIC or needs immediate relief, a surgeon may create a vesicostomy—a small surgical opening in the lower abdomen that allows urine to drain directly into a diaper constantly. ^[1][5] Later, complex urinary reconstructions may be performed.
• Managing Kidney Function: Nephrologists monitor the glomerular filtration rate (GFR) to track kidney health. If the kidneys eventually reach End-Stage Kidney Disease (ESKD) (sometimes referred to as kidney failure), a kidney transplant may be necessary. For many children with PBS, a transplant is a highly effective way to restore long-term health. ^[6][7]

Strengthening the Abdominal Wall

Abdominal reconstruction, or abdominoplasty, is done to provide support for internal organs, improve the child’s ability to cough, and enhance appearance. ^[8][9]

• The Monfort Procedure: This is a specialized, “gold standard” surgical technique for PBS. The surgeon reshapes and tightens the existing abdominal muscles, tucking away the excess skin while preserving blood supply and strength. ^[8][10]
• Benefits: Beyond appearance, a stronger abdominal wall helps with pulmonary clearance (coughing up mucus) and reduces chronic constipation by providing the core pressure needed for bowel movements. ^[11][9]

Physical Therapy and Motor Milestones

The lack of abdominal muscle profoundly impacts physical development. Babies rely heavily on their core muscles to roll over, sit up, crawl, and walk.

Children with PBS often experience significant delays in these motor milestones. ^[12] Physical therapy (PT) is an essential part of daily life. Physical therapists teach parents how to support their child’s core and use alternative muscle groups to achieve mobility. With PT and eventual reconstructive surgery, the vast majority of children with PBS do learn to walk and be active.

Correcting Undescended Testes

Because the testicles in boys with PBS almost always remain inside the abdomen (cryptorchidism), surgery is required to move them into the scrotum to support future hormone health and potential fertility. ^[13][14]

• Fowler-Stephens Procedure: Because the testicles are usually located very high in the abdomen, doctors often use this specialized, staged surgery. The surgeon first clips the main blood supply to the testicle to force it to develop “back-up” blood flow, and then moves the testicle down several months later. ^[1][3]
• Fertility Outlook: While it was once thought that men with PBS could not have children, modern medicine has shown that many produce sperm, and successful fertility treatments have been reported. ^[15]