Surgery-rigged pulley repairs severe facial birth defects

WASHINGTON -- To cure a severe birth defect that left Alyssa Parente with a sunken nose and hugely bulging eyes, Dr. John Polley sliced apart all the bones of her face -- but didn't re-join them before sewing her up.

Instead, 10-year-old Alyssa got a promising alternative to grueling bone grafts: Polley attached a pulley-like contraption to her facial bones that her parents tightened each day with a screwdriver, slowly pulling out the sunken bones so they could grow into the right position.

Two months later, Alyssa had a normal face.

"We're letting the body do its own bone-grafting," explains Polley, who pioneered the technique with a colleague at Chicago's Rush Craniofacial Center.

About 5,000 children a year are born with severe craniofacial defects such as Apert's syndrome, which struck Alyssa, and similar disorders. Their bones fuse together abnormally so the head and face can't grow, leaving sunken faces and misaligned jaws. It's not just a problem of appearance. Often, the malformed head causes problems with eating, breathing, hearing and vision.

Standard reconstruction involves multiple surgeries to cut apart most of the face bones, move them up, down or forward, and then hold them in the new spot with grafts from hip or rib bones. Some children have such little bone to work with that they can't complete such surgery until they're teen-agers. And if the graft is poorly placed, it can't be moved.

'She didn't flinch'

Enter Polley's alternative. It sounds traumatic. After all, Alyssa ran around for weeks last summer with all the bones in her face broken.

"It freaks you out to think we are moving her face," her mother, Eileen Parente of Frankfort, Ill., says of learning to tighten the screws. "But it was something we shouldn't have even worried about. She didn't flinch."

Indeed, Polley's experience with a few dozen patients suggests this so-called "rigid external distraction" can be easier than standard surgery, is done at younger ages, and lets the doctor gently push and pull until the child's face looks just right.

The procedure is still experimental, cautions Dr. Harold Slavkin of the University of Southern California, the National Institutes of Health's former chief of dental and craniofacial research. But it has "enormous promise," he says -- so much that some scientists are studying how to pair this grow-your-own-new-bone method with genetically engineered drugs to make that bone grow faster.