LOS ANGELES (February 4, 1999) -- Joanna Vaughn, 22, a student at Spartanburg Methodist College in South Carolina, is within a few months of completing a medical process that started in 1994 and has added nearly 12 inches to her height and four inches to the length of her arms.
She is one of about 14 patients successfully treated to date at Cedars-Sinai Medical Center for the medical condition called achondroplastic dwarfism, in which the limbs and stature are disproportionately short. For the past seven years, medical geneticists and Joseph Isaacson, M.D., orthopedic surgeon, have specialized in a limb-lengthening procedure developed in Spain.
Although limb lengthening is performed at other institutions throughout the country, the technique employed at Cedars-Sinai is particularly appropriate in treating patients with dwarfism because it lengthens both thighs at the same time and both lower legs at the same time -- unlike the older procedure, the Ilizarov technique.
"The Ilizarov technique is a good technique for doing just one side of the body," said David L. Rimoin, M.D., Ph.D., holder of the Steven Spielberg Family Chair in Pediatrics and director of the Medical Genetics-Birth Defects Center at Cedars-Sinai. "But the Ilizarov technique uses large, external fixators to hold and stretch the leg. Because of this, it is not possible to put them on both thighs at the same time. To get around this, doctors would do one thigh at the same time they were doing the opposite shin, or the thigh and shin of the same leg. But the problem is that if you have to stop for any reason, the patient ends up with legs that are very asymmetrical."
Using the Vilarrubias approach developed in Barcelona, both lower legs are treated at the same time, then both upper arms, followed by both thighs. Patients typically gain 10 to 12 inches in height and about four inches in the length of their arms.
While the operations are no more painful than other orthopedic procedures, the entire process lasts two to three years and "takes a lot of commitment on the part of the patient and the family," according to Dr. Rimoin. Patients spend about three days in the hospital for each operation, during which the bones to be lengthened are broken and external metal rods are attached by pins. For the next three or four months, the patient or a family member turns adjusters -- three times a day -- that continue to separate the gap in the bone by about 1.2 millimeters a day. New bone grows to fill in this space.
"This procedure is undertaken after the age of 12 to allow patients themselves to help make the decision with the parents," said Dr. Rimoin. "It requires a very motivated individual and a supportive family who can help them through the physical problems." Another reason to wait is that if the operation is performed on a younger child, only a few inches in height may be gained, requiring the procedure to be repeated later.
Dr. Rimoin said Cedars-Sinai's is one of only a few programs nationwide specializing in limb-lengthening operations for patients with dwarfism. "I think it's very important that these procedures be done in a center where personnel are familiar with the various problems related to the disorders," he said. "For example, most people with dwarfism have difficulty with sway-back. This can lead to decreased spinal canal volume, which leads to early onset of spine disease, which can eventually lead to sciatica and paralysis. To avoid this, we lengthen and cut the tendons and ligaments in the groin, which alleviates the sway-back and increases the volume in the spinal canal. We believe this prevents or significantly delays the onset of back problems."
In Joanna Vaughn's case, surgery was performed on her lower legs in June of 1994, eventually resulting in a six-inch increase in height. Her upper arm surgery, done in August of 1996, increased her arm length by four inches. She has gained another five or six inches in height as a result of femur surgery performed in April of 1998. She expects to have the fixators -- which currently bear most of her weight and prevent it from resting on her thighs -- removed from her upper legs sometime this spring.
Joanna said that in addition to correcting medical problems, the operations have enabled her to do things most people take for granted: driving a car without having the pedals extended, wearing clothes off the rack, without needing to have them altered. After having her arms lengthened, she was able to put her hands in her pockets. "Just little simple things," she said.
"When trying to get something from the counter, I used to hop up on the counter," she said. "Now I can just reach and get it. You don't realize the things you can accomplish until you have the extra inches."
In addition to providing better overall results, Dr. Rimoin said the Vilarrubias technique is less painful than the older approach. "Ilizarov thought that having patients walk immediately and frequently increased the healing process. But it also causes significant discomfort," he said. "We keep them off their legs during the treatment on their shins, and we have them just intermittently bear weight while their thighs are being treated. I think this approach is far less painful and results in far fewer complications."
"It has not been painful for me at all. But when people look at the fixators, they say, 'That looks so painful.' It's really not," Joanna said. "The only discomfort I've ever had was if the bars have gotten cold. It has just made my legs kind of achy. Any pain, excruciating pain? There's none." Joanna said she and her parents learned about the procedure from a television news story. Discussions about her participation began in 1992 but she chose to wait until after graduating from high school to begin the process.
"It has been a long haul, but worth it. If I had it to do over again, I would in a heartbeat," said Joanna. "It's good that they wait until you're a little bit older and you really realize what you're getting into. It teaches you patience but in the long run, life is going to be a whole lot easier."
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