Twenty orthopedic surgeons in five states delivered the same awful news to Melissa Arnold: The best way to fix her young son Adam's short thigh bone was to cut off his leg and fit him with a prosthesis.
"It was never an option for me," said Arnold, a freelance writer from Red Oak, Iowa, who sought medical opinions from Minnesota to Colorado. "I was quite certain that something like a short leg had to be fixed with something other than an amputation. It's too short, so you cut it off?"
It took years of searching, but Arnold finally found another treatment. Rather than amputate Adam's limb, surgeons in Baltimore would gradually lengthen it - by using its own healing power.
In an era of high-tech medicine, when doctors can implant small defibrillators in patients' chests and transplant entire organs, the mechanics of limb-lengthening admittedly seem crude. The surgery involves drills, a chisel and a mallet - and doctors break the bone in the name of fixing it.
But the results, in some cases, are astounding: Patients can gain anywhere from a few inches to a foot or more in limb length.
"I can't imagine what my life would be like if I had to strap my leg on every morning," said Adam Arnold, now 15, whose 1998 lengthening surgery gained him about six inches.
Dr. Dror Paley, an orthopedist at Sinai Hospital in Baltimore who performed Adam's operation, has done more than 5,000 lengthenings since 1987 (the process usually includes reconstructing deformed knee and hip joints or correcting club feet).
He contends that the majority of children with the most common birth defects that cause unequal leg lengths are losing their limbs unnecessarily.
"While amputation is something that any orthopedic surgeon knows how to do, it is a bit of a cop-out procedure, because what it's saying is, 'I don't offer reconstruction,' " said Paley, co-director of Sinai's International Center for Limb Lengthening, which performed more than 500 lengthenings last year.
"I don't think it's fair that it's the only option they're getting," he said, referring to amputation. "The problem right now is lack of training in these newer technologies, lack of availability [of surgeons], lack of knowledge about how good the results are."
Only a handful of centers in the United States specialize in limb lengthening.
About one in 50,000 children is born with so-called PFFD, or proximal femoral focal deficiency, in which the thigh bone isn't fully developed. Another one in 40,000 has fibular hemimelia, in which the fibula, one of the lower leg bones, is improperly formed or missing altogether.
Sometimes, the deformities leave children with one foot closer to being level with the opposite knee than to the ground. That was the case with Mike Malloy's daughter Jennifer, 10, whose perfectly formed left foot was amputated at 11 months.
"A foot does you no good unless you can get it on the floor and use it," Malloy, a network technician from Ohio, remembers the surgeon saying.
A lengthening can be costly, time-consuming and painful. Some patients require multiple surgeries because it's best to add length in stages, about three inches at a time.
To make it happen, surgeons drill into the bone and insert a series of pins above and below the spot where they plan to fracture it. The pins connect to an external frame resembling scaffolding that holds the bone in place during healing.
By turning a bolt slightly each day, the bone - which is living tissue - is gradually teased apart. New bone grows and fills in the gap. At first, it's the consistency of toothpaste, but after several months, it's as hard as the bone around it.
Orthopedists have not fully embraced the procedure as standard treatment. Just adding length, they say, doesn't necessarily make the surgery a success. When it's not performed correctly, children can end up with fractured bones and joints that pop out of place.
"It has to be pain-free, it has to move well and it has to really work for the kid like a leg," said Dr. Tony Herring, chief of staff at Texas Scottish Rite Hospital for Children in Dallas.
Herring's hospital offers patients with limb deficiencies both options, amputation or lengthening, depending on the severity of the deformity. But, in many cases, he views amputation as a better option.
"The amputation is not really some sort of defeat," said Herring. "That can, for a lot of kids, be a very, very good solution to a problem of great complexity if you go the other way with lengthenings and repositionings."
Some of his patients with prosthetic limbs have played collegiate sports. A high school basketball player who had a double amputation below the knee gained height with his prosthetics: With a new set, he shot from 5-10 to 6-3.
Paley doesn't dispute that many patients with prosthetic limbs can run sprints, ride skateboards and grab rebounds. But, to him, that isn't proof that amputation is the best answer.
"It's only proof that modern prosthetics are so good," he said.
Discovered in Russia
A Russian doctor stumbled onto the idea of limb lengthening by accident in the 1950s. The late Dr. Gavriil A. Ilizarov noticed that many World War II veterans had leg fractures that had never healed. Intending to ease the bone back together, he created an external frame much like the one used today.
But a patient mistakenly turned the rod in the wrong direction, increasing the size of the gap the doctor meant to close. Ilizarov found, though, that new bone was filling in the hole.
"It's millimeter by millimeter - even less than that," said Ilizarov's daughter, Dr. Svetlana Ilizarov, an ex-surgeon who trained in limb-lengthening techniques under her father. Now, she specializes in rehabilitating patients who undergo lengthening at the Institute for Limb Lengthening and Reconstruction at New York's Hospital for Special Surgery.
When she first came to the United States, she was surprised that amputation was routinely performed on children with certain limb defects. Said the institute's director, Dr. S. Robert Rozbruch: "It's easier to do an amputation, but it's not always necessary."
He doesn't promise miracles. His goal for patients who choose lengthening isn't better function than they'd get with an amputation and prosthesis. Rather, it's at least an equivalent result. All of his patients, he said, have been offered amputations by one, two, even three or more surgeons.
"The bottom line is that most people would really rather hold onto their own leg," said Rozbruch. "Most people are even willing to tolerate a little bit less function for the trade-off of maintaining their own leg."
In operating on some patients, Rozbruch concedes that he feels like he's bumping up against the limits of what modern medicine can do. "Sometimes I'm wondering whether I can even do this. Is this even going to work?" he said.
Such was the case with a boy from Sierra Leone whose leg became so infected after a snakebite that he was missing a chunk of bone. After months of rehabilitation following two lengthenings that gained him nearly eight inches, he's now on the soccer team.
Mother has no regrets
For all the complications along the way, Melissa Arnold has never regretted opting for lengthening over amputation, not even when one Midwest surgeon suggested the operation would cripple Adam.
Adam underwent three initial surgeries at the Maryland Center for Limb Lengthening and Reconstruction at Kernan Hospital, where Paley worked at the time. The surgeon fixed deformities of Adam's knee and hip. For a time, the boy was trapped in a cast stretching from his chest to his toes.
After the lengthening, Melissa Arnold diligently rotated the small, square bolt on the outside of Adam's brace four times a day and cleaned the pin holes running up and down her son's leg to stave off infection. "It's not just a surgery, it's a process," she said.
But a few months later, shortly after his brace came off, Adam's pain was unbearable. His femur had fractured where one of the pins had been inserted and was bowing as a result. During surgery to fix it, the bone snapped, and doctors had to insert a metal rod to keep it in place.
Adam still has a rod in his leg. But the only other remnants of his surgery are scars. Adam may ultimately need another lengthening; at 5-4, he's still growing, and his left leg is two inches shorter than his right.
Herring says Paley takes an aggressive approach to lengthening.
"He's doing some kids that we would say, 'Gee, I'm not sure that's a good idea,' " said Herring. "And that's what people on the forefront of things do. They kind of find the boundaries for you."