A move to lengthen limbs internally rather than via an external scaffold could reduce the effects of painful treatments, especially among younger patients
Image: Dr. John Herzenberg
Screaming woke Lanz Ellingsworth. The piercing cries were loud, they were shrill ? and they were coming from his daughter's bedroom.
At 8:30 p.m., Ellingsworth and his wife had tucked their youngest, Lindsay, in for the night. They read her a bedtime story, kissed her on both cheeks and crept out of the room. Six hours later, their little girl was a mass of quivering agony.
In the middle of the night Lindsay had shifted slightly in her sleep. The abnormally brittle femur in her left leg splintered into multiple pieces. She woke from her dreams and plunged into a living nightmare.
"When I woke up that night, what I kept repeating was that 'somebody hit me with a sledgehammer,' Lindsay says. "That's how it felt. Somebody literally smashed my leg with something. And I just screamed."
Lindsay, a tiny, freckled eight-year-old, was used to pain. She was born with a congenital limb deformity called "underdeveloped femur condition," in which one leg is shorter than the other by more than two centimeters. Up to 100,000 people are diagnosed with limb-length disorders in the U.S. each year, according to Bart Balkman, vice president of Ellipse Technologies, an Irvine, Calif.?based orthopedics company.
Left untreated, the condition can result in chronic pain, loss of mobility, degenerative arthritis and debilitating scoliosis, an abnormal and painful curvature of the spine that typically progresses throughout one's lifetime. At birth or shortly thereafter parents whose children have a severe discrepancy (estimates range from a two- to three-centimeter or more differential) must make a decision?amputate the shorter limb or commit to a lengthening regimen to equalize them.
"Can you ever imagine having a child and loving that child and being told that part of your child's leg had to be amputated?" says David Hootnick an orthopedic surgeon in Onondaga County, N.Y., who studies congenital abnormalities. "Can you imagine the emotional stress you'd have to go through?"
Limb-lengthening technology has progressed significantly from the early 1950s when a handful of doctors first began the practice. Gavriil Ilizarov invented the external fixator in Russia, a device he modeled after a horse harness. Now, about 100 surgeons in the U.S. perform limb-lengthening procedures, and a new internal approach offers patients expanded mobility and promises fewer complications. The device, called "Precice," received U.S. Food and Drug Administration approval for consumer use but is still relatively unknown within the greater medical community.
[View a slideshow of the Precice technology.]
By far the most common procedure for children with leg discrepancies?and the one used in Lindsay's surgery?is an external "fixator." Surgeons break a patient's bone in half and pull it apart, allowing the body's natural healing capabilities to fill in the gap with new bone, thereby lengthening the limb.
John Blanco, a specialist in pediatric orthopedics and scoliosis at the Hospital for Special Surgery in New York City, has been performing the operation for years. With the patient under a general anesthetic, Blanco drills screws into the top and the bottom of the afflicted bone. The screws are fastened into a metal frame that will stabilize the leg during and subsequent to the procedure. After cutting a small incision in the leg, Blanco takes a hammer and breaks the bone in two. It's a dull sound, he says, like hitting wood?more of a thwack than a snap. The finished product resembles an Erector Set construction covering the leg.
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