Riley Children’s Health is among one of the few pediatric hospitals to treat the rare Sirenomelia disease, a severe condition causing a fusion of the legs at birth. Investigated under a thorough care process by a team of experts at Riley Children’s, patient Harper Gray is making her way to normalcy.
Also known as mermaid syndrome, the extremely rare disorder affects one in 100,000 babies and involves problems with the lower part of the spine. The condition is diagnosed in the pregnancy stage, and while the cause is unknown and likely fatal within a few days of birth, chief of plastic surgery at Riley Children’s, Gregory Borschel, MD, took lead in the unique and incredibly unforeseen case.
Upon birth in May 2021, Harper’s melding of the legs required collaborative care from the neonatal intensive care unit, as well as extensive support from the pediatric orthopedic program and pediatric urology program at Riley Children’s. While born with only one tiny kidney in addition to mermaid syndrome, the multiple specialists ensured Harper had no breathing problems and proper brain development prior to surgery.
Following an initial colostomy surgery, Dr. Borschel performed zig-zag incisions using flaps, as recommended from conversations with international surgeons in Canada and Italy. Dr. Borschel also made sure the nerves in her legs were working properly.
“One of the goals for Harper is to get her walking,” Dr. Borschel said. “If we do get her walking, we have to maintain the mobility on the knees, and we want to avoid contractures around the knees.”
“We had to make sure she had a blood supply to both legs and then, if the legs were separated, we also had to ensure that the blood supply would be sufficient enough,” Dr. Borschel added. “We got some special images to really look at the blood flow.”
The successful April 2022 procedure lasted seven hours and required an abundance of stitches across the newly de-fused legs.
Post-separation surgery, patients will likely experience fragile leg bones and will require additional surgeries for abnormalities in other parts of the body. While Harper is predicted to walk on her own soon, her diagnosis is not yet finished, and her next steps will require further combined expertise from specialists at Riley Children’s.
“I feel very happy with the outcome so far,” Dr. Borschel said. “She’s not totally done yet. She has club feet; we’ll get that treated too.”
Alongside Dr. Borschel’s leading-edge efforts, orthopedic surgeons, neonatologists and urologists contribute to the ongoing success of mermaid syndrome and more. Learn more in our annual report.