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1/61. Fracture reduction and deformity correction with the hexapod Ilizarov fixator.

    A configuration for the Ilizarov external fixator with six distractors and 12 ball joints in the form of a hexapod was developed. The system allows for six degrees of freedom bone fragment displacement by controlling the distractors. Using this assembly, universal three-dimensional corrections or reductions are possible without the need for complicated joint mechanisms. The device was used in 16 patients: five had displaced tibial fractures with severe soft tissue damage, 10 had deformities or pseudarthroses subsequent to treatment of tibial fractures, and one had an axis deviation in the course of tibial lengthening. Translational (to 40 mm) and rotational deformities (to 33 degrees) were corrected. Final radiographic examinations after the correction procedure was complete showed median residual deformities of 3.5 mm (range, 0-5 mm) and 1 degree (range, 0 degree-4 degrees) in the anteroposterior projection and of 1.5 mm (range, 0-6 mm) and less than 1 degree (range, 0 degree-9 degrees) in the lateral projection. The construction is a useful and important addition to the Ilizarov fixator system. As a bone fixation device it is unique in that its optimal use depends on the availability of computer software.
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2/61. SPONASTRIME dysplasia: report of an 11-year-old boy and review of the literature.

    SPONASTRIME (SPOndylar and NAsal changes, with STRIations of the MEtaphyses) dysplasia is a rare, autosomal recessive bone disorder first described by Fanconi et al. [1983: Helv Paediatr Acta 38:267-280]. Radiographic findings include abnormal vertebral bodies with age-dependent changes, and striations of the metaphyses, scoliosis, and retarded ossification of the carpal bones. Physical features include severe short stature, lumbar lordosis, midface hypoplasia, frontal bossing, and a depressed nasal root. To date, 12 patients from 6 families have been reported. Four additional patients have been reported with a variant of this condition, which includes mental retardation. We report on an 11-year-old boy with features consistent with SPONASTRIME dysplasia. Height was 106.1 cm (-6 SD). He had a coarse appearing face with a depressed nasal bridge, short, upturned nose, and midface hypoplasia. intelligence was normal. A clinical evaluation at 6 years of age suggested the diagnosis of spondyloepiphyseal dysplasia (SED). However, genetics evaluation at 11 years of age with repeat radiologic studies revealed delayed carpal ossification (-4 to -5 SD), metaphyseal irregularities and striations most notably in the distal femurs and the proximal tibias, lumbar lordosis, narrow interpedicular distances of the lumbar spine, and pear-shaped vertebral bodies. These findings were most consistent with the diagnosis of SPONASTRIME dysplasia, and not SED. Although radiographic findings of SPONASTRIME dysplasia are distinguishable from SED, the physical appearance may be similar. Many bone dysplasias have overlapping radiographic findings and clinical presentation but with different recurrence risks, making genetic counseling a challenge.
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keywords = tibia
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3/61. New mesomelic dysplasia with absent fibulae and triangular tibiae.

    We report on two unrelated, sporadic cases of a mesomelic dysplasia characterized by absence of fibulae and severely hypoplastic, triangular-shaped tibiae. Moderate mesomelic shortness was present in the upper limbs with proximal widening of the ulnae. There was also axial skeletal involvement in both cases, characterized radiographically by an abnormal pelvis and marked bilateral glenoid hypoplasia. These cases appear to represent a new form of mesomelic dysplasia distinct from those previously delineated.
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keywords = tibia
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4/61. Spectrum of Schwartz-Jampel syndrome includes micromelic chondrodysplasia, kyphomelic dysplasia, and Burton disease.

    Follow-up and re-evaluation of four patients originally described as examples of severe infantile "micromelic chondrodysplasia" resembling Kniest disease, "kyphomelic dysplasia," and "Burton skeletal dysplasia" revealed the diagnosis of Schwartz-Jampel syndrome (SJS, myotonic chondrodysplasia) in all of them. SJS may be suspected in neonates with Kniest-like chondrodysplasia, congenital bowing of shortened femora and tibiae, and facial manifestations consisting of a small mouth, micrognathia, and possibly pursed lips. The disorder must be differentiated from the Stuve-Wiedemann syndrome, a genetically distinct myotonic chondrodysplasia with similar clinical but different skeletal changes and an unfavorable early prognosis. The demise of "kyphomelic dysplasia" as a nosological entity reemphasizes the symptomatic nature of congenital bowing of the long bones.
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keywords = tibia
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5/61. Unilateral late-onset tibia vara associated with bilateral proximal femoral growth disturbance in monozygotic twins: case report.

    Clinical and histopathological similarities and rare association of Blount's disease with various proximal femoral physeal affections (i.e. adolescent coxa vara and slipped capital femoral epiphysis) are well known. association of tibia vara with another epiphyseal disease of the proximal femur has not been reported previously. In this paper, a monozygotic set of twins with concordant bilateral epiphyseal growth disturbance of the proximal femur and unilateral late-onset tibia vara is presented. Radiological characteristics of the affected knees revealed a wedging in the proximal tibial epiphysis, depression of the medial joint surface and varus deformity of the tibia. Proximal femurs of both cases showed aspheric congruity, coxa magna, shortness of the femoral neck, and subchondral cystic changes. The presented cases support the genetic etiology of tibia vara, and association of the two conditions is unique.
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6/61. magnetic resonance imaging findings and treatment outcome in late-onset tibia vara.

    Several retrospective studies have shown the usefulness of magnetic resonance imaging (MRI) in the evaluation of tibia vara. With respect to the late-onset type, however, there have been no English-language reports on MRI findings. In this article, the authors report on the MRI features of three patients with late-onset tibia vara, the results of treatment that was chosen on the basis of the findings, and the histologic characteristics of the disease. Fat-suppressed MRI revealed partial closure of the tibial physis in one patient and near-closure in another patient. They underwent osteotomy with resection of the bone bridge and have shown no evidence of recurrence. In the third patient, only irregularity of the physis was found on MRI, and she was treated conservatively. Her varus deformity subsequently improved, although it persisted. Histologic examination revealed disorganization and misalignment of the physeal zone and transverse alignment of the trabeculae. The patient with a bony bridge also had discrete ossification centers in the physeal zone.
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keywords = tibia
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7/61. Blount's disease: a lesser known cause of bowlegs mandating early differentiation from physiological bowing.

    We present a 16-month-old child, with progressively increasing bowing of legs, having a normal serum calcium, phosphorus and alkaline phosphatase level; lower limb radiographs revealed bilateral medial tibial metaphyseal beaking. Based on these findings, a diagnosis of Blount's disease (infantile tibia vara) was made and an orthotic management program was instituted for the child.
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keywords = tibia
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8/61. A probable new type of osteopenic bone disease.

    A probable new type of osteopenic bone disease in two sisters and one female cousin is described. In infancy, the radiological findings were osteopenia, coxa vara, periosteal cloaking, bowing of the long bones, and flaring of the metaphyses. During growth, spinal pathology developed with compression of the vertebral bodies and scoliosis in one girl and kyphosis in another. All three children had genu valgum and two developed severe S-shaped bowing of the tibiae. growth was stunted. Inheritance of this disorder is probably recessive. Type I and III collagen biosynthesis was normal. This condition is probably a hitherto undescribed form of osteogenesis imperfecta type III or a new bone disease.
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keywords = tibia
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9/61. Grebe-Quelce-Salgado chondrodystrophy: prenatal diagnosis of two new cases in unrelated families in Southern brazil.

    Grebe-Quelce-Salgado chondrodystrophy is an autosomal recessive non-lethal skeletal dysplasia. Affected individuals have normal head, neck, and trunk skeleton, relatively normal humeri and femora, short and deformed radii, ulnae, tibiae, and fibulae, and severe abnormalities of hands and feet. polydactyly is frequent. Digits present as globular appendages and are characteristic of the condition. The disease is caused by a missense mutation in the gene encoding cartilage-derived morphogenetic protein-1 (CDMP-1). Most cases described in the literature are from brazil and, among these, all had ancestors from a particular region in the state of Bahia. We describe two cases of Grebe-Quelce-Salgado chondrodystrophy visualized by prenatal ultrasound. The patients presented in this report do not descend from the population of Bahia and, to our knowledge, case two is the only case with prenatal clinical diagnosis in a family with no previously affected children.
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keywords = tibia
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10/61. CD99 positive adamantinoma of the ulna with ipsilateral discrete osteofibrous dysplasia.

    An adamantinoma is a rare, low-grade malignant, osteolytic bone tumor occurring predominantly in the diaphysis of the tibia. Osteofibrous dysplasia has been suggested as a precursor lesion to adamantinoma. Evidence for the relationship between these two tumors is based on their similar histologic features, immunohistochemistry, shared clonal abnormalities, overlapping skeletal distribution, and simultaneous occurrence in the tibia and fibula. The ulna is an unusual site of involvement by adamantinoma and osteofibrous dysplasia. Simultaneous involvement of the ulna by adamantinoma and ossifying fibroma has not been previously reported. A case is presented of an adamantinoma of the distal ulna with unique pathologic features occurring with an ipsilateral discrete focus of osteofibrous dysplasia as additional evidence of the relationship between these two lesions.
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keywords = tibia
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