A Big Picture Approach to Treating Posterior Urethral Valves
Nothing about the treatment of posterior urethral valves is easy or straightforward. The experience of Max Flores and his family demonstrates the importance of a multidisciplinary team working together to address all aspects of a child’s immediate needs and long-term care.
Max Flores, now four years old, was diagnosed with obstructive uropathy during a prenatal ultrasound. A maternal-fetal specialist in his hometown provided initial interventions, including placement of a shunt to drain the bladder. These procedures were not wholly successful, and the mother developed amhydramnios. When the specialist shared a pessimistic view of Max’s chances for survival, Max’s parents, Jenny and David, sought a second opinion at Cincinnati Fetal Center, which is located at Cincinnati Children’s Hospital Medical Center.
There, following extensive testing, a team of approximately seven physicians from various pediatric specialties met with the Floreses to review test results, give a realistic appraisal and discuss treatment options. “We suspected that the child had a severe presentation of posterior urethral valves, and saw a high risk of pulmonary hypoplasia and renal failure,” says Pramod Reddy, MD, director of the Division of Pediatric Urology at Cincinnati Children’s. “We also thought that, if he survived, he eventually would need a kidney transplant. At the same time, we believed that with very focused care, we could potentially achieve a positive outcome.”
Multiple diagnoses Max was born by Caesarean section at 35 weeks gestation at nearby University of Cincinnati Medical Center, and immediately transported to the neonatal intensive care unit at Cincinnati Children’s. His left kidney was non-functioning, and his right kidney was dysplastic but producing urine. Testing confirmed PUV, and revealed sequalae of chronic kidney disease (CKD), anemia of CKD and hypertension. Reddy incised the PUV via cystoscopy; a week later, in a separate procedure, he performed a right ureterostomy, bringing the right ureter to a cutaneous stoma to drain urine continuously. The Floreses returned home with Max one month after he was born.
Reddy and David Hooper, MD, medical director of kidney transplantation at Cincinnati Children’s, saw Max frequently in clinic to monitor his condition and provide treatment as needed. “From the beginning we were preparing for a renal transplant, and doing our best to preserve Max’s quality of life long term,” Reddy says. “Urology and nephrology share the same location at the hospital, which allows for real-time consults between the two services. Our entire team is focused on maintaining the health of the bladder pre- and post-transplant, because we know that if the bladder fails, the transplanted kidney will as well.”
Two novel therapies Max’s bladder was hypertrophied and at high pressure as a result of fetal obstruction, and his right kidney function continued to decline. In August 2016, at two years of age, he underwent a pre-emptive living related transplant from his father (the left, non-functioning kidney was not removed). During this phase of care, Reddy performed two novel therapies. The first involved a Botox injection to the bladder prior to transplant, in order to relax the musculature and improve the compliance of the bladder to make it a safe reservoir to connect a transplanted kidney. The second took place after the new kidney was transplanted. Reddy used Max’s preserved right ureter to create a distal ureterostomy, creating a “pop-off mechanism” for the bladder. Max would be able to urinate normally, but the mechanism created a channel to divert pressure from the bladder, thereby relieving pressure on the transplanted kidney.
A high quality of life After transplant, Max was able to void spontaneously for the first time in his life. About a year after transplant, he developed hydronephrosis in the transplant kidney and was placed on intermittent catheterization. He is currently off catheterization and voiding spontaneously with close surveillance to ensure the safety of his transplant kidney, his bladder and his overall health.
“While Max will require ongoing care for life, he has traveled far from being a critically ill preemie to a healthy, happy and active four year old who is enjoying preschool,” Reddy says. “The impact that PUV has may never be curable, but by working hand in hand with nephrology, transplant specialists, nursing and other members of the care team, we can provide true multidisciplinary care that preserves the child’s overall health, their renal-bladder function and achieves the best quality of life possible.”
Later this year, Cincinnati Children’s will open a Posterior Urethral Valves Center – the first of its kind in the world. The new center formalizes the hospital’s long-standing commitment to provide comprehensive, multidisciplinary care for patients with this disorder. Learn more about the center by emailing Pramod.Reddy@cchmc.org
