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The University of Chicago Medicine - Comer Children's Hospital

Surgeons Provide Life-Saving Care for Child with Rare Urologic Condition

When Sandie* visited her local obstetrician in Florida for a 20-week ultrasound, the milestone appointment unexpectedly began the fight of her son's life.

The test detected a problem with the baby's development. The then-mother-of-two remembers the panic of that appointment vividly. "The doctor was telling me that something was wrong, and the thoughts that were going through my head were just terrible," Sandie said.

"The doctor was telling me that something was wrong, and the thoughts that were going through my head were just terrible," Sandie said.

From left to right: Pediatric urologist Mohan Gundeti, MD, baby Jayden and his mom, Sandie.

At birth, physicians diagnosed her son Jayden with cloacal exstrophy, a condition that prevents normal growth and development of the pelvic bones and abdominal organs, mainly the bladder and large bowel. Jayden immediately required an emergency stabilizing surgery to keep him alive through an ostomy, an abdominal opening created for waste drainage. 

Cloacal exstrophy is rare; only one in 250,000 newborns is affected. Most physicians treat one or two cases in their entire career. After extensive online research and speaking with other physicians, Sandie decided the University of Chicago Medicine Comer Children's Hospital physicians Mohan Gundeti, MD, and Robert Bielski, MD, were the right choice to treat her infant son.

Dr. Gundeti, a pediatric urologist, and Dr. Bielski, a pediatric orthopaedic surgeon, teamed up to tackle the challenges of Jayden's condition. Dr. Gundeti is an internationally renowned expert in complex, meticulous urologic repairs, and he has more than a decade of experience treating children with cloacal exstrophy.

"Not everyone can take care of these patients, but we have the experience to complete a repair that will provide the optimal outcomes,"
said Dr. Gundeti.

Drs. Gundeti and Bielski were faced with performing a complex reconstruction of the bladder and genitals, as well as closing a large abdominal wall defect. Additionally, Jayden was 15 months old, and normally this repair is performed in earlier months after the initial stabilization with an ostomy. The longer a child lives with the deformity, the stiffer the body becomes.

"We were happy to have Jayden at UChicago Medicine," Dr. Gundeti said. "There are many complications associated with this congenital condition, and the surgical repair is a very technically demanding procedure that requires extensive insight into the process. Not everyone can take care of these patients, but we have the experience to complete a repair that will provide the optimal outcomes." 

Robert Bielski, MD, is an expert in pediatric orthopaedic surgery at Comer Children's.

Jayden's pelvic opening extended to the surface of the skin, where the soft organs of his abdomen were extended out through the front of his body. In order to repair this defect, he needed several surgeries. 

"Think of the pelvis as a giant ring," Dr. Bielski said. "Normally, its two halves lock together in the front, but in cloacal exstrophy, the halves separate, and the ring opens up." 

During Jayden's complex surgery, which lasted for nearly eight hours, Dr. Bielski realigned his pelvic bones; Dr. Gundeti closed Jayden's bladder, repaired his hernia, moved his testes back into their proper position, closed the abdominal wall defect, and addressed ostomy needs to enable waste to leave his body. Jayden was outfitted with an external fixator device to hold his pelvis in the proper position until it completely healed. Urology resident Vig Packiam, MD, also assisted with the procedures.

Sandie was immensely grateful for the care and support her son received from the Pediatric Intensive Care Unit nurses during the nearly month-long stay at Comer Children's. 

From left to right: Mohan Gundeti, MD; with Sandie and her son Jayden, as well as two nurses from Jayden’s care team, Krista Kirnbach, BSN, RN; Roshini Singh, BSN, RN.

Additionally, administrative assistant Nancy Kozlar and advanced practice nurse Ashley Wilkens connected Sandie with the on-campus Ronald McDonald House when she needed a place to sleep away from her son's hospital bed. They guided her through the insurance approval process as well.

Due to his external fixator, Jayden couldn't lie at more than a 30-degree angle, which meant he couldn't safely sit in a car seat. He had to be transported to and from the Ronald McDonald House by ambulance, which was not covered by insurance. Lisa Kuntz, a social worker at Comer Children's, worked with the ambulance company to give the family a reduced rate, and arranged for UChicago Medicine to pay a portion of the remaining cost.

When Sandie told Kuntz about how much she missed her husband and children still in Florida. Kuntz worked with Southwest Airlines to get the family complimentary airline tickets to Chicago and to get Sandie and Jayden home after his hospital discharge.

"It was great she was able to benefit from our services and did not have to manage without them," Kuntz said. "Because Sandie was so put-together and independent, you might not think she would need help. But I try to think about it from the caregiver's perspective, and I wonder, ‘What would make me feel better if I were in her shoes? What resources do we have to address those needs?'" 

Jayden has healed well from his surgery and is back home in Florida with his parents, his big brother and sister, and his new baby brother. He will need additional surgeries as he grows to maintain his urinary continence, but he is well on his way to a full recovery, thanks to the life-saving, complex care he received at Comer Children's. 

"He still has a long road ahead of him," Sandie said, "but we always stay positive."

*Last names withheld for privacy