| | | |  | | | | | | Dror Paley, M.D.
Associate Professor of Orthopedic Surgery, University ofMaryland Medical Center, Baltimore | | | | | | YES Individuals with achondroplasia are at a significant handicap when negotiating everything from coat racks to public phones to a car's gas pedal. Short arms make it dangerous to reach across a stove range and hard to turn on a water faucet. Also, they have a significant limb-to-body disproportion. It affects them psychologically, socially, and emotionally. Short stature also makes them less competitive in many jobs.Despite these limitations, many individuals with achondroplasia compensate extremely well, and the majority lead productive lives. Surgical techniques, derived from the Ilizarov technique, are now available to safely and significantly alter height for a functional improvement and to improve body proportion and image. This in turn leads to a positive psychosocial effect. These surgical techniques have rapidly improved, with circular and monolateral fixators, lengthening over nail to shorten treatment times, and, most recently, a fully implantable lengthening nail with no external fixator. We can now lengthen lower limbs by 10 to 12 inches and upper limbs by four to five inches, with excellent radiographic and functional results. Most complications can be prevented or treated, with no permanent sequelae. Stature lengthening involves three separate procedures-for both tibiae, humeri, and femora. It's best to start in childhood, as early as 6 years and preferably by 12. It's more taxing for older patients. Patients are usually finished by the age of 16. If one starts early, patient height can remain parallel to peers'. Based on these results, stature lengthening for dwarfism should be available at specialized centers. | | | |
| | | | |  | | | | | | Charles I. Scott Jr., M.D.
Chief, Division of Medical Genetics, Alfred I. duPont Institute Wilmington, Del.; Chairman, Medical Advisory Board, Little People of America | | | | | | NO The Little People of America's medical advisory board believes extensive limb lengthening is experimental for dwarfs, though it can be helpful for nondwarf children born with one short leg. The LPA and 16 health-care professionals on its medical board believe that limb lengthening is inappropriate for achondro-plastic dwarfs. Shortness is a cosmetic problem. One may want height, but no one dies of shortness. Long-term effects of bone lengthening aren't known. Nor is there proof that it lessens all the orthopedic or neurological problems inherent in dwarfism. Achondroplastic dwarfs are prone to back problems-severe pinching of the nerves and spinal cord. Being made taller doesn't lessen this. Patients can develop oste-omyelitis or have nonunion. Besides bone, soft tissues, arteries, nerves, and veins also have to be lengthened. And calves are never as full and well developed cosmetically as among unoperated dwarfs. To our knowledge, only one U.S. group does lengthening under a strict protocol, studying the patient and family psychologically before, during, and afterward. It also studies the effects on body chemistry, collagen, bone, blood flow, and nerve conduction. This group has operated on fewer than a dozen patients in the past four years. It does not operate on all comers, monitor by taking a few x-rays, and then publish results-too often the case. Little people feel leg lengthening merely creates a taller dwarf. Bodies are disproportionate, feet tiny, fingers short, and they still have a propensity for spinal-cord compression.So until long-term results are known, the LPA's medical board recommends it be done only in special circumstances. | | | |
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