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1/5. A 'made in one piece' skeleton in a 22-year-old man suffering from sickle cell anaemia.

    A 22-year-old African male with known sickle cell anaemia was referred by a Congolese medical centre with a request to improve his poor physical condition. He was unable to walk, stand or sit because his large joints and his spine were either ankylosed or very rigid. Radiographs showed joint fusion from the third to the fifth cervical vertebrae, of both hips, of the left knee, and a bilateral osteonecrosis of the humeral head. There was no scintigraphic evidence for an active osteomyelitis (99mTc-MDP (methyldiphosphonate) bone scan, Tc monoclonal antigranulocyte scan and 99mTc sulphur colloid scan). To improve his mobility the right femoral head was resected in June 1997; 14 days later the left femoral head was resected. Four months after the resection of the right hip, a right uncemented total hip prosthesis was implanted on this side. One month later the same type of hip arthroplasty was performed on the left side. During the postoperative rehabilitation period the patient regained autonomy. We have found no previous reports of such severe and multiple joint complications in a single patient suffering from sickle cell anaemia.
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2/5. High tibial osteotomy with use of the Taylor Spatial Frame external fixator for osteoarthritis of the knee.

    BACKGROUND: High tibial osteotomy (HTO) is used to treat medial compartment osteoarthritis of the knee in active patients with varus alignment. In this study we review the clinical and radiographic outcomes associated with the Taylor Spatial Frame (Smith & Nephew), and its use in HTOs, and we include an illustrative case report. methods: In 7 patients with medial compartment osteoarthritis of the knee and varus alignment, the Taylor Spatial Frame was applied to the tibia in the operating room and a proximal tibial osteotomy was performed. patients followed a computer-generated turning schedule until the desired correction was achieved. The frame was removed when the osteotomy site had healed. The lower extremity measure (LEM) was used to assess physical function. Clinical outcome measures relating to the Taylor Spatial Frame included latency, time to correction, time in the frame, number of residual corrections and complications. Radiographic outcomes included preoperative Resnick grades of osteoarthritis, pre- and post-correction limb alignment and tibial slope measurements. RESULTS: Average (and standard deviation) LEM grade at a mean 41 (14) months follow-up after correction was 94% (5%). Average latency was 8 days, time to correction was 15 days, time in the frame was 23 weeks and number of residual corrections was 1.3. Complications were similar to those for external fixators. Radiographic correction goals were met in all patients. CONCLUSION: The Taylor Spatial Frame is a valuable asset when using HTO to treat medial compartment osteoarthritis of the knee.
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3/5. Bilateral nonunited femoral neck fracture in a child with osteopetrosis.

    We report a case of osteopetrosis with bilateral nonunited femoral neck and coxa vara in a 7-year-old boy. There was a history of traumatic transcervical left femoral neck fracture unsuccessfully treated by internal fixation. We performed a bilateral subtrochanteric valgus osteotomy. K-Wire fixation failed on the right side due to hardness of the bone. Finally, internal fixation with an angular plate after predrilling of the femoral neck offered stability. Bone union was achieved on both sides, resulting in full recovery of normal physical activity.
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4/5. Bejel in Malatya, turkey.

    Bejel is a form of endemic syphilis which is transmitted by non-venereal route. According to our knowledge, no cases of bejel have been reported from turkey for more than 30 years. In this report, three children and their father who suffer from bejel are presented. The history, physical findings, laboratory investigations, treatment and results of the cases are reviewed, and the related literature is discussed.
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5/5. Focal scleroderma in children: an orthopaedic perspective.

    Focal scleroderma is a connective-tissue disorder manifested by fibrosis of the skin and subcutaneous tissues. Consequently, it may be associated with joint contractures, extremity deformity, and impairment of extremity function. It has a variable clinical course, with both remissions and recurrences. In resistant cases of extremity involvement, treatment considerations should include physical and occupational therapy, medical treatment, bracing, serial casting, and surgical intervention. We reviewed seven patients treated between 1960 and 1990 with significant joint contractures secondary to focal scleroderma. Serial casting was found to be useful for contractures about the wrist, knee, and ankle. Soft-tissue release was an effective treatment for contractures at the wrist, hip, and ankle. knee disarticulation was performed on one patient with a severe knee-flexion contracture. Epiphysiodesis and femoral shortening osteotomy were effective treatments for leg-length equalization in a case of hemiatrophy.
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