Boy undergoes rare surgery to correct severe scoliosis

by: April Frawley Birdwell

A 4-year-old Ocala boy born with a severe form of scoliosis became one of the first children in the Southeast to receive a titanium rib device that will allow his rib cage to expand as he grows, said University of Florida surgeons, who performed the two-and-a-half-hour operation in September at Shands at UF.

The device spreads vertically to expand his chest cavity, creating more room for his organs while correcting his scoliosis. Unlike adolescents who develop scoliosis, children born with the disease usually have ribs that have fused, preventing their chest cavities from growing even as the organs inside them continue to enlarge, said Raymund Woo, MD, UF College of Medicine associate professor of orthopaedic surgery and chief of pediatric orthopaedics, who led the procedure.

Prior to the development of the titanium rib -- a thin bar that can be manually adjusted so the rib cage can expand over time -- many children with severe congenital scoliosis died as their bodies outgrew their fused ribs, Woo said.

Only about 1,000 children in the United States have this severe form of congenital scoliosis with fused ribs, Woo said. The main problem these children face is that their fused ribs constrict lung development, said George H. Thompson, MD, Case Western Reserve University professor of orthopaedics and director of pediatric orthopaedics.

“The lung really gets most of its development in the first eight years of life,” said Thompson, who is also president of the Scoliosis Research Society.

Shamar Honor, who has been Woo’s patient since he was about 6 months old, received two of the titanium ribs. Woo had to separate some of the child’s fused ribs so they could be attached to his rib cage. The only parts of the vertical ribs that actually touch the boy’s bones are the points where they are attached, Woo said.

Woo will not know how well the device works until Shamar’s scoliosis worsens or improves, he said.

The titanium ribs have additional length inside of them, which will allow Woo to expand them by making small incisions over the spots where the device is attached to Shamar’s rib. He will have to come back every six months to have them adjusted.

Because the surgery is so unusual, Woo received special training. An orthopaedic resident assisted in the surgery and Vincent Deeney, MD, a University of Pittsburgh orthopaedic surgeon who has performed the surgery before, was in the room to offer guidance. Another team was also in place to monitor Shamar’s spinal cord, Woo said.

The U.S. Food and Drug Administration approved the device, produced by the Paoli, Pa., company Synthes, for humanitarian use in 2004. Aside from correcting scoliosis and allowing the rib cage to expand, the device will also allow Shamar to maintain flexibility, something that older scoliosis patients typically sacrifice when rods are fused into their spines.

For more information or to refer a patient, call 352-273-7001.

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