Extensive limb lengthening in Ollier s disease: 25-year follow-up

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1 861 Extensive limb lengthening in Ollier s disease: 25-year follow-up Clinic of Traumatology and Orthopedics, Tartu University Clinics, Estonia Key words: Ollier s disease, limb lengthening, cartilage replacement, long-term follow-up. Summary. A case of extensive lower limb lengthening (32 cm) in a 14-year-old male patient with Ollier s disease is reported. A varus deformity of the femur and a valgus deformity of the tibia were evident. The femur was successfully lengthened 22 cm by metaphyseal distraction, and the tibia was lengthened 10 cm by two-stage distraction-compression method with a cylindrical bone allograft. Ilizarov s distraction device was used. Radiologically, a good bone regenerate was formed. Host bone has incorporated (like sarcophagi) the allograft of tibia. No evidence of vascular or neural disturbances was found. The lengthening indices were counted for femur 22.5 days per centimeter and for tibia 21 days per centimeter, altogether 15.5 days per centimeter. Bone lengthening was performed through the Ollier s disease foci. Fine needle biopsy investigation showed that most embryonic cartilage cells had been replaced with bone tissue. After five years and a 25-year follow-up the patient was satisfied with the result. The function of the knee joint was limited, but the limb was fully weight-bearing. Signs of knee osteoarthritis were found. Introduction In 1900 L. Ollier, a French professor of orthopedic surgery and one of founders of French orthopedics first described the condition known today as Ollier s disease (1). Ollier s disease (dyschondroplasia, multiple enchondromatosis) is a rare non-hereditary disorder of unknown pathogenesis. Dyschondroplasia is a developmental affection rather than a neoplastic disease (2). It is characterized by multiple, asymmetrically distributed, intraosseous cartilaginous foci and subperiosteal deposition of cartilage with a common pattern of unilateral involvement of the lower extremities. The femur, tibia, and ilium are the bones most often involved. Also, the phalanges and the metatarsals are frequently involved (3). The tumors are located in the epiphysis and the adjacent parts of the metaphysis and the shaft. Deformities resulting from the tumors include shortening caused by lack of epiphyseal growth, broadening of the metaphyses, and bowing of the long bones. An occasional lesion has the characteristics of both fibrous dysplasia and enchondromatosis (4). Limb-length inequality is not only a cosmetic problem. Shortening of a lower limb affects the vertebral column and causes stiffness in the ankle joint of the shortened leg. A significant shortening of the limb always causes gait disturbances due to massive and inconvenient orthosis, and leads to a decline in working capacity even up to the development of disability. Therefore, correction of extensive limb shortening remains a topical problem in orthopedic surgery (5 9). Despite the long treatment time and usually complicated course the patients are satisfied with the results even if the cosmetic appearance is not the best. They do well socio-professionally (10, 11). Different options of treating Ollier s disease have been used starting with amputation (12) in suspected malignancy with severe deformity of the limb due to intraosseous cartilaginous foci. Limb lengthening (2), either gradual bone grafting (13) or callus distraction (14), is the most common method of correcting deformities in the lower limb. Successful treatment depends on efficient interdisciplinary efforts of health care professionals from medicine, nursing, physical and occupational therapy, recreational therapy, and social services. Long-term consideration must address the potential for malignant changes (1, 2). Case report A 14-year-old male patient was hospitalized at the Department of Orthopedic Surgery of Tartu University Hospital in October 1978 with the diagnosis of Ollier s disease. The anatomical shortening of the right lower limb constituted 32 cm 22 cm of the thigh and 10 cm of the leg. A varus deformity of the femur and a valgus deformity of the tibia were evident. The function of the knee joint was limited; the extension-flexion range was 0 /0 /90. Radiological investigation Correspondence to A. Märtson, Clinic of Traumatology and Orthopedics, Tartu University Clinics, Puusepa 8, Tartu, Estonia. aare.martson@kliinikum.ee

2 862 showed focal areas of the cartilage in metaphyseal parts of the long bones and in the pelvis. The histological investigation performed after a fine needle biopsy of the femur showed evidence of embryonic cartilage cells. The patient used limb orthosis (Fig. 1, 2). In November 1978, metaphyseal distraction was started after distal femoral corticotomy, performed by special chisel through Ollier s focus. Ilizarov s external distraction device was used. The knee joint remained unfixed. Distraction was done asymmetrically, more from the medial side to correct the varus deformity. The rate of distraction was 1 mm per day, divided into four equal parts each after 6 8 hours. Post-operative rehabilitation started immediately after the operation. After eight months, a 22 cm lengthening of the femur (75% of the initial length) was achieved. Radiologically, a good bone regenerate was formed. No evidence of vascular or neural disturbances was found. The fixation of the femoral segment continued during 16 months. In September 1979, a two-stage lengthening of the tibia was started. Osteotomies from the middle of the tibia and fibula were performed. An intramedullary nail was inserted, and Ilizarov s distraction device was applied. The external fixator remained unremoved on the thigh. Distraction rate was 1.5 mm daily. After 2 months, a 10 cm lengthening of the leg was achieved. In the second stage the bone defect was filled with a cylindrical bone allograft (Fig. 3). The fixation of the leg continued for two months. During this period, formation of osteoperiosteal regenerate around the bone graft was evident. The distraction devices were removed both from the thigh and from the leg in April 1980 (1.5 years after the beginning of femur distraction). The lengthening indices were counted for femur 22.5 days per centimeter and for tibia 21 days per centimeter (altogether 15.5 days Fig. 1. Before the treatment A clinical appearance, B patient with orthosis and C-x-ray.

3 Extensive limb lengthening in Ollier s disease: 25-year follow-up 863 A B Fig. 2. The histological examination A cartilaginous Ollier s foci, B cartilaginous Ollier s foci replaced by bone tissue after limb lengthening. Fig. 3. At the end of the distraction A patient in Ilizarov s devices, B femur, C tibia (bone allograft has been placed).

4 864 Fig. 4. After the treatment (right lower limb lengthening) A patient, B femur, C tibia; host bone has incorporated the allograft. per centimeter) (Fig. 4). Fine needle biopsy investigation showed that most embryonic cartilage cells had been replaced with bone tissue. A five-year follow-up showed a relatively good functional result. The patient had only a few complaints. He had a weight-bearing limb with fully corrected inequality of limb length. The range of knee extension-flexion was 0 /0 /60. The follow-up after 25 years (in 2004) confirmed the same functional results but with a painful knee. Host bone has incorporated the allograft (like sarcophagi). Signs of osteoarthritis were observed on the knee radiograph (Fig. 5). Discussion The pathogenesis of Ollier s disease is unknown. E. Aegerter divided the proponents of the hypotheses of the formation mechanism of enchondromas into two groups: those who attribute multiple enchondromas to ectopic rests of chondroblasts, and those who attribute enchondromas to failure of chondrocytes and the growth plate to mature (12). Several reports have dealt with correction of limb length inequality and deformity correction at the same time (6, 11, 14). Opinions vary with regard to the amount of one-time lengthening, way and level (7, 8) of osteotomy, rate of distraction and stabilization of bone fragments, and radiological evaluation of bone regeneration (6, 9, 11). Good results have been reported using distraction bone lengthening after metaphyseal corticotomy (5 8, 11, 14). We performed corticotomy using a special curved corticotome elaborated in our clinic. However, other means using drill holes, chisel and Gigli s saw are available, too (7). We believe that a waiting period after corticotomy, lasting 5 7 days, is more rewarding than achievement of immediate distraction. In the reported case, we also relied on the efficiency of corticotomy through the focus of Ollier s disease, as encouraged by R. Pandey with coauthors (14). We observed that the formation of the bone regenerate and its quality was good, which was

5 Extensive limb lengthening in Ollier s disease: 25-year follow-up 865 Fig. 5. Clinical and x-ray result in 25 years later A patient, B femur, C tibia; host bone has incorporated the allograft (like sarcophagi). demonstrated by fine needle biopsy after the lengthening (Fig. 2). The duration of fixation with Ilizarov s external distraction device was acceptable and even beyond our expectations. Lengthening indices 22.5 and 21 days per centimeter were gained for femur and tibia respectively. In different studies the lengthening indices vary from 0.74 months per centimeter to days per centimeter in single-level corticotomies (9, 11) and 0.57 to 1 month per centimeter in doublelevel osteotomies/corticotomies (7, 8). It was also interesting that despite Ollier s disease we did not observe delayed regeneration on the level of femur; the formation of osteoperiosteal regenerate on the tibia proceeded at the usual rate. The fracture through cartilaginous foci in Ollier s disease heals usually well (3). It showed that in this young patient bone regeneration was kept on good level. R. Pandey with co-workers wrote in their report that their experience suggested that surgeons need not fear defective mineralization in callus distraction in Ollier s disease. Rather, bone formation improved the local deficiency of bone mineralization (14). The time of removal of the external distraction device has been discussed, too. The most common method of monitoring limb lengthening is plain radiography (9). We did not use any other special methods in this case. We predicted a long fixation period with the external device, which was confirmed by the current clinical case. No psychosocial problems concerning limb lengthening were detected (10, 11). The problem of development of osteoarthritis in neighboring joints remains in limb lengthening. Development of knee osteoarthritis was moderate with a few complaints. No signs of malignancy were observed although as it may occur in up to 50% of cases (4, 12). Conclusion The patient was satisfied with the treatment result. Therefore, extensive limb lengthening through Ollier s disease foci could be indicated in the case of a signi-

6 866 ficant shortening of the lower limb. Development of osteoarthritis and transforming Ollier s foci into malignant tumor should be checked during the follow-up. Acknowledgement We wish to thank Mr. Enn Veldi, docent of English from the University of Tartu for revising the manuscript. Žymus galūnės pailginimas sergančiajam Ollier liga (25 metų stebėjimo duomenys) Tartu universiteto klinikų Traumatologijos ir ortopedijos klinika, Estija Raktažodžiai: Ollier liga, galūnės pailginimas, kremzlės pakeitimas, ilgalaikis stebėjimas. Santrauka. Aprašomas žymus apatinės galūnės pailginimas (32 cm), atliktas 14 metų pacientui, kuriam diagnozuota Ollier liga (dauginė kaulų chondromatozė). Akivaizdūs varus tipo šlaunikaulio ir valgus tipo blauzdikaulio iškrypimai. Šlaunikaulis sėkmingai pailgintas 22 cm distrakcija metafizių srityse, o blauzdikaulis 10 cm dviejų stadijų distrakcijos-kompresijos metodu panaudojant cilindrinį kaulo transplantantą. Naudotas Ilizarovo distrakcijos aparatas. Rentgenologinis tyrimas parodė, kad susiformavo tvirtas kaulo regeneratas. Šeimininko kaulas priėmė šlaunikaulio transplantantą. Nepastebėta jokių kraujotakos ar neurologinių sutrikimų požymių. Apskaičiuoti pailginimo rodmenys: per 22,5 dienos šlaunikaulis pailgėjo 1 cm, per 21 dieną šlaunikaulis pailgėjo 1 cm (vidutiniškai per 15,5 dienos pailgėjo 1 cm). Kaulų pailginimas atliktas Ollier ligos židinių vietose. Biopsijos tyrimas parodė, kad dauguma embrioninių kremzlės ląstelių pakeistos kauliniu audiniu. Po penkerių ir 25 metų pacientas buvo patenkintas gydymo rezultatais. Kelio sąnario funkcija ribota, tačiau atraminė kojos funkcija normali. Pastebėti kelio osteoartrito simptomai. Adresas susirašinėti: A. Märtson, Tartu universiteto klinikų Traumatologijos ir ortopedijos klinika, Puusepa 8, Tartu, Estija. El. paštas: aare.martson@kliinikum.ee References 1. Little C. Ollier disease: an interdisciplinary approach. Orthop Nurs 1994;13: Tachdjian MO. Pediatric orthopedics. 2nd ed. Philadelphia: Saunders; p Bessler W, Grauer W, Allemann J. Case report 726. Skeletal Radiol 1992;21: Carnesale PG. Multiple enchondromatosis (Ollier disease). In: Canale ST, editor. Campbell s operative orthopaedics. 10th ed. St. Louis: Mosby; p Bonnard C, Favard L, Sollogoub I, Glorion B. Limb lengthening in children using the Ilizarov method. Clin Orthop 1993;293: Glorion C, Pouliquen JC, Langlais J, Ceolin JL, Kassis B. Femoral lengthening by callotasis. A study of a series of 79 cases in children and adolescents. Rev Chir Orthop Reparatrice Appar Mot 1995;81: Kristiansen PL, Steen H. Reduced lengthening index by use of bifocal osteotomy in the tibia: comparison of monofocal and bifocal procedures with the Ilizarov external fixator. Acta Orthop Scand 2002;73: Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop 1990; 250: Saleh M, Goonatillake HD. Management of congenital leg length inequality: value of early axis correction. J Pediatr Orthop B 1995;4: Dutoit M, Rigault P, Padovani JP, Finidori G, Touzet P, Durand Y. The fate of children undergoing bone lengthening in congenital hypoplasia of the legs. Rev Chir Orthop Reparatrice Appar Mot 1990;76: Stanitski DF, Bullard M, Armstrong P, Stanitski CL. Results of femoral lengthening using the Ilizarov technique. J Pediatr Orthop 1995;15: Mitchell ML, Ackerman LV. Case report 405: Ollier disease (enchondromatosis). Skeletal Radiol 1987;16: Urist MR. 37-year follow-up of multiple stage femur and tibia lengthening in dyschondroplasia (enchondromatosis) with a net gain of 23.3 centimeters. Clin Orthop Relat Res 1989;242: Pandey R, White SH, Kenwright J. Callus distraction in Ollier s disease. Acta Orthop Scand 1995;66: Received 25 April 2005, accepted 24 August 2005 Straipsnis gautas , priimtas