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4 Things to Know About Bladder Exstrophy at Nationwide Children’s Hospital

4 Things to Know About Bladder Exstrophy at Nationwide Children’s Hospital 1024 734 Abbie Miller

Led by V. Rama Jayanthi, MD, and Peter Cuckow, MB, BS, FRCS, the Bladder Exstrophy Program at Nationwide Children’s is poised to launch a new era of care for children in the United States.

When Drs. Jayanthi and Cuckow start talking about their new program, their excitement is palpable.

“We’re so excited to bring this level of care and treatment to the United States, and to central Ohio,” says Dr. Jayanthi, chief of Urology at Nationwide Children’s. “By adding Dr. Cuckow and his expertise in the Kelly procedure to our team, we’re opening the door for many families to experience their best possible outcomes.”

V. Rama Jayanthi, MD (left), and Peter Cuckow, MD (right)

The new Bladder Exstrophy Program at Nationwide Children’s is built around a comprehensive philosophy of care and the Kelly procedure, which uses existing tissue and muscle to create a ring of muscle that acts as a sphincter. The Kelly procedure is part of a large reconstructive surgery to address the structural and functional needs of patients with bladder exstrophy. This surgery has not been widely available in the United States.

In addition to performing surgeries, Dr. Cuckow, who is an attending at Nationwide Children’s and a renowned exstrophy expert with decades of experience in the United Kingdom, will train a new generation of exstrophy surgeons in the approach. This training component further strengthens the team-based, comprehensive care approach the two envision for their patients.

“It’s not just a single surgery that makes our program unique and leads to excellent outcomes, it’s our whole strategic approach to caring for children and families with bladder exstrophy,” says. Dr. Cuckow.

1. Bladder exstrophy is a rare, treatable, complex disorder of development.

Bladder exstrophy is a rare condition when a baby’s bladder sticks out through the wall of the abdomen. The bladder is flat and can be seen on the outside of the body; the skin, muscles and pelvic bones don’t correctly connect; and the bladder can’t hold urine.

Beyond the urinary tract, bladder exstrophy affects the digestive and reproductive systems. The baby often has defects of the abdominal wall, bladder, genitals, pelvic bones, rectum and anus.

Children with bladder exstrophy also have vesicoureteral reflux. This condition causes urine to flow the wrong way — from the bladder back up into the ureters. Children with bladder exstrophy also may have epispadias. Epispadias is a condition where the urethra does not fully close during development.

“One of the first things we share with families during a consultation is that bladder exstrophy is not something to be afraid of,” says Dr. Cuckow. “We show them how to care for their baby after birth and before surgery, enabling the families to get comfortable with the condition, accept it, and feel confident caring for their child.”

2. Bladder exstrophy care begins at diagnosis – even if that’s before birth.

According to Drs. Cuckow and Jayanthi, about 50% of bladder exstrophy patients are diagnosed before birth. By partnering with the Fetal Center at Nationwide Children’s, the bladder exstrophy team, which includes surgeons, nursing staff, psychologists and others, will meet with families before birth so that a plan is in place.

Parents are often relieved to hear that they don’t need to travel to a special hospital to deliver a baby with bladder exstrophy, says Dr. Cuckow. They can give birth in their community hospital and take the baby home. That newborn period of bonding is vitally important for parents and baby – and it’s not necessary to disrupt it.

Even if bladder exstrophy isn’t diagnosed until after birth, it’s important for families and providers to know that emergency transfer is not needed, though an early visit with the bladder exstrophy team will support and educate new parents.

“Patients come in every situation,” says Dr. Jayanthi, chief of Pediatric Urology at Nationwide Children’s. “Whether they are coming for diagnosis after a suspicious ultrasound, after delivery with a new diagnosis or later in life, we’re here to help them. Sometimes we see older children who have had surgeries already but aren’t experiencing the quality of life they’d like to achieve. We are often able to use our comprehensive approach to help them.”

The strategic framework of the Bladder Exstrophy Program enables personalized medicine and the ability to meet each patient and family where they are.

3. Bladder exstrophy with the Kelly approach does not require osteotomy.

“Our team follows a strategic, two-phased approach to bladder exstrophy repair. The first step is surgical closure, which puts the exposed bladder back inside the abdomen,” explains Dr. Jayanthi.

The closure procedure is done once the baby is established on feeding and has started to gain weight – usually between 2 weeks and 3 months of age. During this time the family have become bonded and familiarized with the reality of bladder exstrophy.

After the closure and a brief hospital stay, the child will go home with the family until it is time for a second surgery, known as the Kelly procedure. The Kelly operation is a more extensive reconstruction of the affected anatomy, addressing issues of bladder control and continence, and reconstructing the urethra, ureters, belly button, penis and other anatomical anomalies. This surgery usually occurs around age 1.

Hospitalization with this approach is dramatically reduced – requiring a week to 10 days inpatient – compared to traditional approaches to bladder exstrophy repair. Notably, the Kelly approach does not require external fixators (casts, braces) because an osteotomy is not part of the plan.

“I haven’t performed an osteotomy in decades,” says Dr. Cuckow. “People may say that we can’t rule them out completely, but in my experience, they are unnecessary.”

4. “Dry by preschool” isn’t just a goal; it’s the reality for most patients treated with this approach.

“With all girls and many of the boys, we hope the Kelly operation will be the last big surgery,” says Dr. Cuckow. “In my experience, it’s true for most – about 75%.”

Some boys may need additional surgery to bring the urethra to the tip of the penis, and in teenage years, some girls may need surgery to their vaginal openings.

Patients who don’t become continent will be offered conventional surgery to make them dry, this could include bladder neck reconstruction, ileocystoplasty and formation of an appendico-vesicostomy. This happens in about 20% of patients.

“Our team will be here to support patients and their families every step of the way,” says Dr. Jayanthi. “As the child grows and matures, needs change. We’re here for all of it.”

“It is always my hope that a child I’ve treated will grow up to forget me,” says Dr. Cuckow. “I know I’ve done my job if the child is able to go on and live a normal life, pursue their hopes and dreams and even start their own families if they choose.”

 

Image credit: Adobe Stock (Header); Nationwide Children’s Hospital (Drs. Jayanthi and Cuckow)

About the author

Abbie (Roth) Miller, MWC, is a passionate communicator of science. As the manager, medical and science content, at Nationwide Children’s Hospital, she shares stories about innovative research and discovery with audiences ranging from parents to preeminent researchers and leaders. Before coming to Nationwide Children’s, Abbie used her communication skills to engage audiences with a wide variety of science topics. She is a Medical Writer Certified®, credentialed by the American Medical Writers Association.

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