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11/61. Juvenile Blount's disease: bilateral case with asynchronous onset.

    We encountered a rare case of bilateral juvenile Blount's disease diagnosed as unilateral at the onset. The patient was a 9-year-old girl who visited our outpatient clinic with pain and deformity in the left knee. Examination of the left knee showed severe varus deformity. Correction of the varus deformity and tibial shortness was carried out at the same time by the callotasis method using an Orthofix external fixator. During follow-up the varus deformity in her right knee gradually progressed. At 18 months after the initial surgery, the right varus deformity and tibial shortness were corrected at the same time by the callotasis method using an Orthofix external fixator; proximal epiphysiodesis was also performed. The coronal-plane alignment of both knees remains good. In cases with bilateral involvement of Blount's disease, the onset of the deformity is usually nearly synchronous. In some cases, however, it begins unilaterally, as with this patient, so it is essential to check both sides by means of tomography or magnetic resonance imaging and not just radiography. Moreover, we must be careful to plan the treatment in such a case so as to anticipate possible leg-length discrepancy problems.
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ranking = 1
keywords = tibia
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12/61. Clinical and molecular analysis of Grebe acromesomelic dysplasia in an Omani family.

    Grebe syndrome is a rare autosomal recessive acromesomelic dysplasia. The syndrome was studied clinically, radiographically, and genetically in an Omani family with four affected children. The affected persons had normal axial skeletons, severely shortened, and deformed limbs with severity increasing in a proximo-distal gradient, and subluxated joints. The humeri and femora were hypoplastic with distal malformations. The radii/ulnae were shortened and deformed whereas carpal bones were invariably rudimentary or absent. The tibiae appeared rudimentary; fibulae were absent in two children, and some tarsal and metatarsal bones were absent. The proximal and middle phalanges were absent while the distal phalanges were present. The father and mother had short first metacarpal and middle phalynx of the fifth finger and hallux valgus respectively. Transition A1137G and deletion delG1144 mutations in the gene encoding the cartilage-derived morphogenetic protein-1 (CDMP-1) were identified in this family. The A1137G is a silent mutation coding for lysine, whereas the delG1144 predicts a frameshift mutation resulting in a presumable loss of the CDMP-1 biologically active carboxy-terminal domain. The affected siblings were homozygous for the delG1144 mutation while parents were heterozygous.
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ranking = 0.5
keywords = tibia
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13/61. The morbid anatomy of congenital deficiency of the tibia and its relevance to treatment.

    Two specimens obtained from a 5-month-old boy with bilateral type 1a congenital tibial deficiency were dissected to describe their morbid anatomy. Examination revealed a rigid equinovarus deformity, absence of a tibial remnant, an abnormal saddle-shaped talus, and several tarsal coalitions. observation of the arterial pattern during surgery supported the previously reported finding that persistence of an immature arterial structure is inherent in this condition. knowledge of potential structural anomalies is essential during the planning of an amputation or of a knee or an ankle reconstruction. Anatomic abnormalities may affect the design of soft-tissue flaps in an amputation and plantigrade positioning and foot biomechanics in reconstructive procedures.
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ranking = 3
keywords = tibia
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14/61. A case with Pyle type metaphyseal dysplasia: clinical, radiological and histological evaluation.

    Pyle type metaphyseal dysplasia is a rare autosomal recessive disease that is primarily affect metaphyses. Here we present a case with Pyle type metaphyseal dysplasia. The characteristic features of the case were metapyhseal broadening with undertubulation and Erlenmeyer flask sign at distal femoral and proximal tibial metaphyses. There were also platyspondyly with biconcave lens appearance of the vertebral bodies, congenital hip dislocation and normal cranium. Bone histopathology showed decreased number of osteoclasts. To the best of our knowledge, this is the first reported case of Pyle type metaphyseal dysplasia from turkey.
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ranking = 0.5
keywords = tibia
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15/61. Hemicondylar tibial osteotomy in Blount's disease. A report of 2 cases.

    Two neglected cases of Blount's disease with severe sloping of the medial tibial condyle and excessive ligamentous laxity are presented. Elevation of the medial tibial condyle gave good results.
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ranking = 3
keywords = tibia
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16/61. Arthroscopic visualization during excision of a central physeal bar.

    A centrally located developmental physeal bar in the proximal tibia was removed via a metaphyseal window. The use of an arthroscope to assist in the complete removal of the bone bridge is described. The arthroscope improved visualization and allowed reduction in the size of the metaphyseal window. At 2-year follow-up, the growth plate showed no evidence of bar reformation.
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ranking = 0.5
keywords = tibia
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17/61. Bone transport with frozen devitalized bone: an experimental study using rabbits and a clinical application.

    We evaluated the feasibility of bone transport with frozen devitalized bone in the tibia of 20 adult female rabbits. A 1.5-cm segmental defect was created, followed by proximal tibial transverse osteotomy to remove a 2.5-cm segment to be transported after devitalization with liquid nitrogen. The proximal and distal tibia and the devitalized autogenous bone each were fixed with two half-pins. After 2 weeks, transport of the devitalized segment was initiated at 1 mm/day. In a control group, callus formed at proximal and distal osteotomy sites, and distracted callus gradually maturated. The docking site fused and the medullary canal reappeared by 8 weeks after completing distraction. In the frozen-bone group, bone formation proceeded from the proximal tibial end, and the distraction callus slowly matured. The transported segment remained nonviable until revascularization proceeded from its periphery, evident 8 weeks after completion of distraction. Docking sites fused well without infection at pin sites. There was no sign of infection around the pin sites of the devitalized bone. We finally describe similar successful treatment of a 13-year-old girl with tibial deformity resulting from osteofibrous dysplasia who was treated successfully with this procedure. Therefore, bone transport using frozen devitalized bone can regenerate living bone. This experimental model represents the development of a new reconstruction technique of bone transport with devitalized bone.
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ranking = 2.5
keywords = tibia
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18/61. New skeletal dysplasia with unique brachydactyly.

    We report on 2 male propositi, their mothers, and a maternal aunt with a new skeletal dysplasia associated with a unique pattern of digital malformation, variable mild short stature, and mild bowleg with proximal overgrowth of the fibula. The digital malformations comprise a pattern of brachydactyly which includes short, abducted thumbs, short index fingers, and markedly short, abducted great toes. The radiographic findings include hypoplastic thumbs and great toes with short first metacarpals and first metatarsals, absent distal phalanges of the index fingers and second toes, and coalescence of the carpal and tarsal bones. Radiographs of the long bones show mild metaphyseal and epiphyseal irregularity, tibial spurs, and relative elongation of the fibulae. The males are very similarly affected whereas the females show phenotypic variation and are generally less severely affected. The family histories from 2 fairly extensive pedigrees suggest X-linked dominant inheritance.
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ranking = 0.5
keywords = tibia
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19/61. A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature.

    adamantinoma is known as a low-grade malignant biphasic tumor. Classic adamantinoma is in general characterized by admixture of both epithelial and osteofibrous components that are associated with various proportions and differentiation patterns. Osteofibrous dysplasia (OFD) is a self-limited benign fibro-osseous lesion of bone during infancy and childhood. OFD-like adamantinoma is characterized by predominance of osteofibrous tissues, in which small groups of epithelial cells are only detected by careful search or immunohistochemistry. There have been controversies as to the potential correlation among OFD, OFD-like adamantinoma and classic adamantinoma. We report an unusual case of adamantinoma arising in the tibia, with an extensive review of the literature. The present findings suggest a direct correlation between OFD-like adamantinoma and classic adamantinoma. At the age of 12 years, the tibial biopsy lesion was diagnosed as OFD. At the age of 23, the lesion became larger and more destructive on x-ray films. The biopsy lesion was diagnosed as classic adamantinoma. Wide excision was performed. The primary lesion was retrospectively diagnosed as OFD-like adamantinoma because of presence of keratin-positive epithelial cells within the stroma. At five years after surgery, the patient was free from recurrence or metastasis. The retrospective histological findings of OFD-like adamantinoma in the original biopsy and of a classic adamantinoma in all sections of the later resection specimen raised the possibility of an unusual progression of OFD-like adamantinoma to a classic adamantinoma. The present case and the literature review suggest that an OFD-like adamantinoma may be a precursor lesion of classic adamantinoma. Therefore, the possibility of progression of OFD-like adamantinoma to a classic adamantinoma should be kept in mind, particularly when the destructive changes are seen radiologically.
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ranking = 1
keywords = tibia
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20/61. Pyle's disease. A description of two clinical cases and a review of the literature.

    The authors present the case of a sister and a brother who came to our observation at different times and both affected with Pyle's disease with marked valgus of the knee. The clinical cases reported demonstrate how in Pyle's disease with significant valgus of the knee temporary asymmetrical tibial epiphysiodesis was able to correct the deformity, not only by provoking the momentary arrest of growth in the growth cartilage on the internal tibial aspect, but also by allowing for bone remodeling in the tibial metaphysis.
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ranking = 1.5
keywords = tibia
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