Long and Short of It: Limb-Lengthening Surgery Changes Lives by Gina Shaw Lansana Aguste Lapia might well have been another child amputee wandering the villages of Sierra Leone. Separated from his family and likely orphaned during the brutal 10-year civil war that killed some 20,000 people, Lansana still loved to do what many boys his age do: play soccer. One day in early 2000, when he was about 6 (heís not sure exactly how old he is), Lansana chased a soccer ball into the jungle and was bitten by a venomous snake. The snakeís venom wasnít the kind that killed, but instead caused ìlocal necrosisîósevere tissue damage. The boy was rescued by villagers who performed a bloodletting, but over the next year and a half, a severe infection developed in the bitten leg, shortening and deforming it. ìItís a wonder that they didnít just amputate the leg,î said Dr. S. Robert Rozbruch of the Hospital for Special Surgery in Manhattan. ìHonestly, under those circumstances, thatís probably what I would have done.î But for some reason, they didnít. And one day in late 2001, Dr. Ian Zlotolow, chief of dentistry at New Yorkís Memorial Sloan-Kettering Cancer Center, came to Sierra Leone on a humanitarian mission. There, he saw Lan sana playing soccer on wooden crutches, and he soon felt such a powerful connection to the determined little boy that he arranged for U.N. officials to bring him to the United States on an emergency medical visa in 2002. Once in New York, Zlotolow brought Lansana to his colleague, Rozbruch. What could be done for the boy? In most places, the answer would have been the same: amputate the damaged limb. But Rozbruch specializes in a rare and complex form of orthopedic surgery known as limb lengthening. The Hospital for Special Surgeryís Institute for Limb Lengthening and Reconstruction is one of only a few in the United Statesóor the worldówith the necessary expertise to perform complicated limb-lengthening surgeries such as Lansanaís. ìHe had a good foot, but it was basically just held on by soft tissue. From the knee down to the ankle, the bottom two-thirds of the tibia was just gone,î said Rozbruch. ìAnd because there was no bone there, his foot had also started to deform. What he had was a very short leg with a soft tissue bridge holding a bone defect that had scarred into place.î Over the next two years, in a series of nine surgeriesówhich is probably not yet overóRozbruch and his colleagues grew about 20 centimeters of bone along Lansanaís damaged leg. ìThe basic principle is that you cut the bone, and while it is healingówhich it does by making new boneóyou can gradually ëdistractí the bone so that it lengthens,î Rozbruch explained. ìYou pull apart the gap in the break youíve just made, and if you create the right biological and mechanical environment, it will stimulate the body to create more bone and fill in that gap.î Today, Lansana lives in San Francisco with his adopted father, Zlotolow. This summer, he visited Rozbruch in New York, and even the expert was stunned by what he saw. ìHeís completely healed. Heís playing soccer and basketball. Itís beyond even my expectations,î Rozbruch said. ìWhen he came in walking with a big smile on his face, I was delighted to see him, but when I saw him run across the room and saw pictures of him playing basketball and soccer, I was just overwhelmed.î Lansana will probably need one more surgery to equalize his leg length as he grows, but ìthe hard partís done,î said Rozbruch. Who Needs Limb Lengthening? Lansanaís case was particularly challengingóRozbruch called it about the most complicated heís ever handledóbut there are thousands of people, both children and adults, throughout the world who seek limb-lengthening surgery. At the International Center for Limb Lengthening at Sinai Hospital of Baltimore, where Rozbruch received his training, ìweíve treated probably patients from over 50 countries to date, and from every state in the union,î said Dr. Dror Paley, co-director of the center. ìIíve been doing this since 1986, and in the last 17 years, my partners and I have probably treated over 7,000 patients with these techniques.î In fact, Paley and Dr. John Herzenberg, the centerís other co-director, were the first team in the United States to dedicate their practices to limb lengthening and deformity reconstruction. Who might need limb-lengthening surgery? Although much attention has focused on the very few patients who seek limb lengthening for cosmetic reasonsómen who would rather be 5í9î than 5í6î, for exampleóthe vast majority of limb-lengthening patients seek the surgery for medical reasons. ìWe see a whole variety of pathologies: patients with congenital deformities, such as one leg thatís three or four inches shorter than the other,î Paley explained. ìThese are typically children, although we sometimes see adults who never got treated as children. When we first started, we had a lot of adults who came out of the woodwork.î Many adult patients today, he observed, come to the center with damaged limbs as a result of trauma from injuries, often in car or motorcycle accidents. Sometimes a break will heal badly, leaving a leg or an arm shortened or bent. Infections after surgery can also cause shortened limbs. ìIn the past, and in some places even to this day, the alternative was either to wear a prosthesis around the leg or more likelyóbecause the foot gets in the wayóto amputate the leg entirely and put on a prosthesis.î Each case is different, said Rozbruch. ìMy typical patient may be someone whoís had a motorcycle accident and sustained an injury to the tibiaómuch like Lansanaís case, only less severe and with less bone missing. Either because of infection afterward or because of bone loss at the time of injury, the patient ends up having a defect in the bone of five centimeters or so, about two inches.î In early July, Rozbruch operated on an 18-year-old high school varsity athlete. ìAbout three weeks before, after a game, he told his mom that he had some pain and swelling in his leg. It turned out to be a bone tumor,î Rozbruch recalled. After surgery at Sloan-Kettering to remove a 16-centimeter cancerous segment of bone, the boy was referred to the Hospital for Special Surgery to have his leg repaired. Some of the other conditions treated by limb-lengthening specialists such as Paley and Rozbruch include achondroplastic dwarfism, severe bowleggedness, Blountís disease (a condition that causes the leg to bow and rotate internally), dropfoot and clubfoot deformities, and knock-knee deformities. From Russia, With Love The technique used by Paley, Rozbruch and other limb-lengthening experts to repair damaged limbs has its origins in mid-20th-century Russia. Surgeon Gavriil A. Ilizarov developed the concept in the early 1950s, after treating many veterans of World War II whose leg fractures had not healed. The first limb-lengthening procedure using what has come to be known as the ìIlizarov methodî was actually a mistake. Ilizarov developed an internal fixation frame that was placed around the leg and designed to compress the fracture to stimulate bone healing. He told his patient to turn an attached rod that would gradually compress the area of non-union in the boneóbut the patient turned the rod the wrong way and separated the fracture. The doctor noticed that new bone had formed in the gap, and he began to research and develop his techniques for limb lengthening. The process must be very finely calibrated. If you pull the bones too far apart too soon, new bone may not form between the broken ends, and soft tissue may become contracted. Pull them apart too slowly, and the bone may consolidate too soon, preventing the lengthening device from pulling it apart further. Surgeons use a variety of lengthening devices, depending on the patientís needs: ìThe most common are external fixators, which are devices that attach to the bone by means of thin wires or thicker pins that have a screw threading at their attachment to the bone,î noted Paley. Doesnít this hurt? Yes, said Paley, but probably not as much as you might imagine. ìA lot of reporting on this surgery has overemphasized and exaggerated the pain involved. If you come sit in my waiting room with 20 or 30 patients, all at different stages of treatment, you wonít be able to pick one out whoís in pain. If this is done well, there shouldnít be a lot of pain.î Limb-lengthening surgery involves two essential phases: the ìdistractionî phase, in which the two ends of the damaged limb are pulled apart and new bone fills in, and the ìconsolidationî phase, in which the new bone is hardening and becoming stronger. ìRight after the distraction surgery, thereís pain,î said Paley. ìAnd during the lengthening process, the consolidation process, thereís usually a mild, dull ache thatís well controlled with moderate pain medication.î Most patients, he added, will say that the worst pain is during physical therapy, which must be done to maintain strength and flexibility in the affected limb. Compared to conventional orthopedic surgery, such as a hip replacement, the pain experience of limb lengthening is exactly the reverse. ìWhen you have a hip replacement, thereís a lot of immediate post-surgical pain, and then the patient gradually feels less pain,î Paley said. ìWith limb lengthening, itís the other way around. The procedure is relatively minor, and thereís some aching from the external device and the pins, which goes away. And then they start doing stretches and the muscles get tighter and tighter. They have to do a lot of physical therapyóin other words, you have to make them worse for a while so they can get better. Once youíve done all that, they enter the consolidation phase, waiting for their new bone to harden.î Future of Limb Lengthening Up until recently, almost all limb-lengthening devices have been external. Recently, a fully implantable device that can be inserted inside the bone has been approved for use primarily in adults and some skeletally mature adolescents. ìThatís changed everything,î said Paley. ìThere are no pins on the outside and a very minimal incision, and the patient doesnít have to wear this big ëbird cage.í Implantable limb lengthening is the wave of the future.î This new device, called the intramedullary skeletal kinetic distractor (ISKD), is a telescopic metal rod inserted into the hollow part of the bone. A screw is implanted at each end to attach the lower and upper parts of the device to the appropriate ends of the bone, while new bone grows in the middle. ìThe device lengthens by the two telescopic pieces rotating relative to each other. If they rotate just a tiny amount, say three degrees, it lengthens,î Paley explained. Any movement of the leg by the patient causes the tubes to rotate, so the patient is causing the leg to lengthen just by doing normal daily activities. Physicians keep track of how much the limb has lengthened each day using a digital monitor that records movement from a magnet inside the device. The device includes an alarm that reminds the patient to monitor daily lengthening. ìIt can be difficult,î admitted Paley. ìOnce youíve done the amount of lengthening you need to do for that day, you need to take it easy, because thereís no way to stop the device. Now that weíve done over 100 surgeries using this device, weíve learned how to teach patients to manage it and gauge their activity. They can also wear a removable plastic brace that limits the deviceís rotation and the rate of lengthening.î The only limiting factor in limb lengthening, said Rozbruch, is the bodyís ability to make new bone. ìSome people make bone better than others, and of course young people tend to generate new bone better than older people do,î he said. ìOur work is fitting in well with a lot of research into bone formation thatís being done by orthopedic and musculoskeletal specialists, trying to isolate the factors that improve bone healing.î ìThis whole field is such an exciting field to be in. We save a lot of legs. We get referred a lot of patients that other people essentially give up on,î said Rozbruch. ìBut every case gives us the opportunity to push the envelope. Youíre never quite sure what is possible.î Gina Shaw is the medical writer for The Washington Diplomat.