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11/26. Cervical instability in skeletal dysplasia. Report of 6 surgically fused cases.

    Six patients with skeletal dysplasias complicated by deformity or instability of the cervical spine were reviewed. Three patients had congenital spondyloepiphyseal dysplasia and 1 each suffered from Morquio's syndrome, pseudoachondroplasia, and chondrodystrophia calcificans congenita. Four patients had myelopathy of varying severity from slight weakness of one leg to tetraplegia. At a mean age of 6 (3-11) years, they underwent posterior fusion and immobilization in a halo brace. In all the cases the extent of postoperative neurologic recovery was, however, limited. Our cases illustrate the need of early diagnosis and fusion of cervical instability.
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12/26. Maxillary growth following total septal resection in correction of orbital hypertelorism.

    A rapid survey of eight cases with orbital hypertelorism did not reveal any evident change in maxillary displacement during facial growth following resection of nasal septum and medial displacement of orbital cavities. Accurate analysis of four cases according to the Ricketts long-term growth forecast showed a reduction of growth in the region of anterior nasal spine and only in the posteroanterior direction. This is not necessarily due to the absence of nasal septum. These patients have such severe deformities and the surgical procedure is so extensive that many factors can be responsible for deficient premaxillary growth. Further studies are needed, particularly in patients who, since 1975, have had the same operative method, but without septal resection, to know whether this more conservative procedure has changed the posteroanterior growth of the premaxilla. However, a nasal bone graft has a good prognosis, even when performed in children.
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13/26. Indications and technique for the operative treatment of hypoplastic deformities of the odontoid process.

    On the basis of four typical cases of congenital hypoplastic deformities of the odontoid process the clinical significance, symptoms, diagnosis and the indications for operative treatment of the malformations are described. It is further shown that these malformations lead to clinical manifestations only when associated with a primarily existing, or a later acquired inadequacy of the ligaments with movement-induced changes in the position of the atlas. The operative treatment recommended involves a special technique for a bilateral dorsal fixation with tibial grafts. By this operative technique a substantial or even completely stable fixation of the atlas can be achieved, in the acute phase by the stability of the tibial grafts and in the long term by the bony fusion which occurs.
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14/26. Traumatic tetraparesis in craniocervical dysplasia. Case report.

    Case report of traumatic induced tetraplegia due to multiple cranio-cervical abnormalities with cord compression at the level of C4/C5. Diagnostic and operative procedure.
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15/26. Rotational acetabular osteotomy for the dysplastic hip.

    A circumacetabular osteotomy of the acetabulum was initially done at the University of tokyo Hospital by one of us (H. T.) in 1968. This procedure, which rotates the acetabulum, was designed to correct a dysplastic acetabulum in adolescents and adults. The surgical exposure combines both an anterior and a posterior approach. Between 1974 and 1982 this operation was performed on 103 patients (120 hips) with acetabular dysplasia, some showing early degenerative arthritis. The forty-five hips (forty-one patients) that form the basis of this report were followed for three years to eight years and ten months (average, four years and six months). Thirty hips showed only acetabular dysplasia, and fifteen were in the early stage of degenerative arthritis. The ages of the patients at the time of operation ranged from eleven to forty-two years, the majority being in the second or third decade of life. All of the forty-five hips had a preoperative center-edge angle of 10 degrees or less, but most of them had a nearly normal value after surgery. In the majority of the hips either limp or pain with exertion, or both, had disappeared, and a satisfactory range of motion had been restored.
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16/26. Orthopaedic problems associated with survival in campomelic dysplasia.

    In the early part of this century sporadic reports of campomelic dysplasia appeared in the literature, along with reports of other entities, under the term "congenital bowing of the long bones." In 1970 Spranger et al. considered campomelic dysplasia a distinct entity. The terms "camptomelic" and "campomelic" have been used interchangeably in the literature since that time. The patient in the present report is a 6 1/2-year-old white girl with classic long-limbed campomelic dysplasia. The patient has had severe respiratory problems throughout her life. As an infant she was treated by a Pavlik harness for subluxation of the hips. She later required two cervical and two thoracic posterior spinal fusions, as well as release of complex foot deformities and osteotomy of the right tibia. Perioperative complications have been significant. Currently, her height and weight are those of a 2 1/2-year-old child, and she has the bone age of a 3 1/2-year-old child. Nevertheless, she is in first grade at a regular school. Certainly, the prognosis for patients with campomelic dysplasia is guarded, but some survivors may be helped by judicious orthopedic care.
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17/26. Craniometaphysial dysplasia with leukoencephalopathy. A case report.

    A case of craniometaphysial dysplasia with extensive degeneration of the cerebral white matter is presented. The cortex of the swollen part of the long bones was quite thin and there was striking trabecular atrophy. Both the vault and base of the skull showed marked thickening and sclerosis, leaving no interlaminal zone. It is probable that the diffuse degenerative change of the cerebral white matter with gliosis bears some resemblance to that produced by a circulatory disturbance of the great vein of Galen. Due to the narrowed foramen magnum, deformed atlase and axis, and the surrounding postoperative scar, the upper cervical cord was compressed, markedly atrophic and degenerated. Other segments of the cervical and thoracic cord displayed secondary wallerian degeneration and focal neurolytic lesions in the white matter. At the level of Th11 there was a pencil-like malacic lesion, suggesting an apparent interference of circulatory disturbance due possibly to the deformed vertebral column.
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18/26. Sacral agenesis with progressive neurological deficit.

    In the past, sacral agenesis was thought to be a form of bony malformation associated with static neurological deficits. The majority of therapeutic recommendations involved orthopedic correction of foot and leg deformities. Myelographic studies were seldom obtained, and surgical exploration was rarely attempted. We recently encountered two cases of sacral agenesis with progressive neurological deficits; both patients had myelographically demonstrable lesions and both responded favorably to surgical treatment. We believe that patients with caudal agenesis can be divided into two groups: one with static neurological deficits that probably would not improve after operation, and the other with progressive neurological findings probably associated with specific neuropathological lesions that would be amenable to operative therapy. We therefore endorse a more aggressive approach toward the management of patients in the second group. Upon the diagnosis of caudal agenesis, a detailed documentation of the base line neurological status, the bladder and bowel functions, and the degree of lower limb deformities should be obtained. A diligent follow-up program is essential to detect progression which, when present, should be followed promptly by myelography and surgical intervention. The etiology, classifications, and clinical features of sacral agenesis are discussed, and the associated neuropathological abnormalities are analyzed.
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19/26. The hip-shelf procedure. A long-term evaluation.

    Renewed interest in the hip-shelf procedure as a solution to the dysplastic acetabulum in the older child stimulated an evaluation of the long-term results in an effort to clarify the role of this procedure. Only patients with a minimum of ten years of follow-up were evaluated in the study. The average follow-up for the entire series of twenty-nine hips in twenty-seven patients was twenty-two years and ten months. At evaluation, pain was present in fifteen hips, a limp was present in twenty-five, and degenerative changes were present in twenty hips. Complications occurred in twenty-one hips and additional operative procedures were required in fourteen hips. Twenty-one of the twenty-nine hips were evaluated by us, with an average follow-up of twenty-five years and eight months. One hip was rated as excellent; eight, as satisfactory; and twelve, as poor. Although the hip-shelf procedure has been described as a safe, conservative, and reliable approach to the problem of a dysplastic acetabulum, our long-term results suggest a more cautious view of the use of this procedure. We recommend consideration of other stabilizing procedures for the dysplastic acetabulum.
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keywords = operative
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20/26. Dynamic axial external fixation in the surgical treatment of tibia vara.

    osteotomy is the well-established treatment of Blount's disease (tibia vara), although the types of fixation used vary considerably. The use of dynamic axial external fixation to stabilize osteotomies for tibia vara until solid union occurs without the use of supplemental casting has not been reported by other authors. From 1985 until the present, we have used osteotomy with dynamic axial external fixation as treatment of 31 tibiae in 23 patients. All osteotomies healed and there was no postoperative loss of correction. There was an average correction of 20 degrees between the pre- and postoperative mechanical axis. Advantages of dynamic axial external fixation include ease of application, adjustability, early weight bearing, the ability to lengthen the extremity, and no second operation for removal of hardware. Based on our results, we believe that dynamic axial external fixation is an excellent form of osteotomy stabilization in the surgical treatment of tibia vara.
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ranking = 2
keywords = operative
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