Urethral Valve Disorder Compromises Kidney Development

Quick Case Study

A boy born with posterior urethral valve disorder experienced chronic kidney disease and bladder dysfunction as a result of the prenatal effects of the blockage.

Immediately after birth, the child had undergone a procedure to clear the obstructive valve tissue—the first of 3 surgeries to help preserve residual kidney function and overall childhood development.

When the patient was 4 years of age, John S. Wiener, MD, Duke’s chief of pediatric urology, performed cutaneous vesicostomy to alleviate the deleterious effects of the abnormal bladder by allowing urine to spill freely onto the skin and into a diaper. The procedure temporarily arrested kidney decline.

Question: Recognizing the patient would eventually outgrow his damaged kidneys, what long-term care strategy did Duke pediatric nephrologists, urologists, and transplant surgeons use?

View case conclusion  

Answer: They employed vesicostomy to allow the patient to grow and develop as normally as possible and delay inevitable kidney failure. At the appropriate time in the child’s development, the Duke team moved forward with plans for kidney transplantation. When the boy was 10 years old, Wiener used a novel balloon catheter to temporarily close the vesicostomy opening to determine if the bladder would safely accept a new kidney and ureter. The procedure worked and allowed the child to urinate normally, so the vesicostomy opening was permanently closed. The following year, the patient received a deceased-donor kidney transplant just 7 days after he was placed on the list for transplant.

The case required a collaborative, multidisciplinary solution, says Eileen Tsai Chambers, MD, medical director of Duke’s Pediatric Kidney Transplant Program.

“Given this patient’s history of urologic challenges, it was a very complex case,” Chambers says. “We were hoping to avoid dialysis and move directly toward transplant.”

Wiener, who had treated the patient since he was a toddler, worked closely with John Foreman, MD, and Shashi K. Nagaraj, MD, former and current chiefs of Duke’s Division of Pediatric Nephology to plan the treatment strategy.

“Cases such as this highlight the success of our collaborative culture, particularly the pediatric nephrology, abdominal transplant, and urology teams at Duke,” says Wiener. “We aren’t just reading each other’s notes. We are stopping and talking to the patients and their families while they are in the clinic. We are working side by side, day by day.”

Bradley H. Collins, MD, led the abdominal transplant team who performed the vascular portion of the procedure while Jonathan C. Routh, MD, a pediatric urologist with a focus on kidney transplantation, implanted the ureter into the overly muscular bladder.

“The urologic aspects of his transplant were challenging,” Routh says. “Patients with posterior urethral valves are at high risk for complications after transplant, so the surgery has to achieve just the right balance—the 'Goldilocks spot,' you might say—to avoid reflux into the kidney while also preventing ureteral blockage.”

Kidney disease in children can take a toll on families, says Nagaraj, who has worked with the family for years. “While we are providing care to this youngster, we are mindful of the toll it takes on the family and siblings,” Nagaraj says. “Our efforts are focused on assisting these parents toward a successful outcome. This child has a family who has provided excellent care, despite setbacks.”

Collins met the young patient before the successful transplant. “He was incredibly upbeat, very optimistic considering all he’s been through,” Collins says.

The average kidney transplant is effective for approximately 15 to 20 years, so it should support the patient into adulthood.