1/67. Jarcho-Levin syndrome: report on a long-term follow-up of an untreated patient. Jarcho-Levin syndrome is a genetically transmitted rare entity characterized by multiple vertebral and rib anomalies. The multilevel skeletal involvement causes short stature, neck and thoracic cage deformities, and restrictive lung disease that is usually the cause of early death. The authors describe a 33-year follow-up of a patient with this syndrome who represents, to their best knowledge, the longest survival of a patient with this entity. ( info) |
| Selective dorsal rhizotomy is used widely as a means of treating spasticity associated with cerebral palsy. Little is known regarding the effect of the procedure on the development or progression of spinal deformity. The authors reviewed six patients with progressive deformity after rhizotomy. Prerhizotomy and postrhizotomy records of physical examinations and radiographs were reviewed retrospectively in an attempt to identify risk factors for development of and/or rapid progression of, spinal deformity. Detailed preoperative and postoperative evaluation of spinal alignment should be undertaken, particularly in those patients who may be at risk of rapidly progressive deformity. ( info) |
3/67. The Camera-Marugo-Cohen syndrome: report of two new patients. This report describes two unrelated patients with obesity, mental retardation, body asymmetry, and muscle weakness. Several obesity syndromes with common characteristics have been described. Findings in our patients, in addition to those of the previously reported cases, include body asymmetry, characteristic physiognomy, lordosis, and typical anomalies of hands and feet. These physical manifestations correspond to the Camera-Marugo-Cohen syndrome. Our patients represent the second and third cases of this condition. ( info) |
4/67. The fate of untreated developmental dislocation of the hip: long-term follow-up of eleven patients. Eleven patients with developmental dislocation of the hip who had no treatment other than observation were followed up for an average of 40 years. Nine of the 11 patients had bilateral hip dislocations. None of the hips developed avascular necrosis. overall clinical and functional results were very satisfactory despite uniformly unsatisfactory radiographs. These patients achieved a better quality of life than have many patients of similar age who have undergone surgical treatments aimed at reducing such late-presenting hip dislocations. ( info) |
5/67. Bilateral hip arthroplasty for ochronotic arthropathy. ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare hereditary metabolic disorder characterised by the absence of the enzyme homogentisic acid oxidase and associated with various systemic abnormalities related to the deposition of homogentisic acid pigment (ochronotic pigment). In this report, we describe a 53-year-old, HLA-B27( ) woman with ochronotic arthropathy. In addition to the typical clinical features of the disorder, she had bilateral hip involvement, which was improved by cementless total hip prosthesis. ( info) |
6/67. Progressive pseudorheumatoid arthropathy of childhood. Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Spranger et al is a rare autosomal recessive disorder. An 11 year-old girl was diagnosed as having PPAC at Ege University, faculty of medicine, Department of Paediatrics. Her complaints of painful joints, difficulty in walking and joint contractures began at the age of 3 years and she was treated for juvenile rheumatoid arthritis for 8 years. Her symptoms did not respond to nonsteroid anti-inflammatory treatment. During her last hospitalisation period, she was reinvestigated. Radiological examination showed spondyloepiphyseal dysplasia, severe acetabular irregularity and osteoporosis. All the laboratory test results for rheumatoid arthritis were negative. The clinical and radiological findings of the patient are illustrated. ( info) |
7/67. Respiratory failure in postpneumonectomy syndrome complicated by thoracic lordoscoliosis: treatment with prosthetic implants, partial vertebrectomies, and spinal fusion. STUDY DESIGN: This study investigated the case of a 17-year-old girl with postpneumonectomy syndrome, complicated by a thoracic lordoscoliosis, who was successfully treated with prosthetic implants, partial vertebrectomies, and anteroposterior spinal fusion. OBJECTIVE: To report a unique case and describe the authors' method of treatment. SUMMARY OF BACKGROUND DATA: Postpneumonectomy syndrome is an uncommon complication of pneumonectomy. Many case reports describe successful treatment with insertion of prosthetic implants into the empty hemithorax to shift the mediastinum to its original position. Thoracic lordoscoliosis reportedly has contributed to pulmonary compromise, but no cases have shown its occurrence in the setting of postpneumonectomy syndrome. methods: The patient was observed at the National Children's Hospital in tokyo, referred to Children's Hospital in los angeles, california for surgical correction, and followed in tokyo for the next year. RESULTS: Two prosthetic implants with an injection port for further expansion were positioned in the right hemithorax to restore the mediastinum to its normal position. Anterior discectomies, partial vertebrectomies, and fusion of T5-T10 was performed concurrently. Then 5 days later, posterior spinal fusion of T1-T12 with instrumentation and bone graft were performed to correct the thoracic lordoscoliosis and increase the chest cavity space. At 1 month after the surgery, the patient was extubated after being ventilator dependent for 5 months. At the time of operation, the girl was ventilator dependent and nonambulatory, but 1 year later could participate in all activities of daily living without any oxygen supplementation. CONCLUSIONS: Postpneumonectomy syndrome can be treated successfully with prosthetic implants to restore the normal position of the mediastinum. Thoracic lordoscoliosis can complicate the syndrome and may be corrected to help restore normal pulmonary function. ( info) |
8/67. Isolated thoracolumbar and lumbar hyperlordosis in a patient with cerebral palsy. A severe isolated thoracolumbar and lumbar hyperlordosis spinal deformity occurring in a patient with cerebral palsy is rare and has not been reported before. The authors describe the presentation, operative considerations, and treatment of patients with this unusual hyperlordotic spinal deformity, particularly those with cerebral palsy. A multiple-stage surgical reconstruction was required to correct this complex spinal deformity. The patient underwent bilateral femoral extension osteotomies along with spinal extensor myotomies to ensure proper prone positioning for his anticipated spinal surgery. Then he had staged anterior releases and spinal fusion from T8 to the sacrum followed by 2 weeks of "90-90" femoral skeletal traction. Finally, a posterior spinal fusion with instrumentation from T2 to the pelvis definitively corrected his deformity. The patient responded well to surgical intervention without complications and continues to have stable correction of his hyperlordosis deformity 2 years after surgery. Severe lordotic sagittal plane spinal deformities can be treated with anterior and posterior spinal fusion and instrumentation with intervening traction in the properly selected and prepared patient who has cerebral palsy. ( info) |
9/67. Extended posture of lumbar spine precipitating cauda equina compression arising from a postoperative epidural clot. We report a patient with nonoperatively treated acute cauda equina compression arising from an epidural clot that developed after decompressive surgery for lumbar canal stenosis. A 43-year-old woman underwent lumbar laminotomy, and was symptom-free for 3 hours; but this was followed by paresis. Postoperative myelography showed obstruction of the contrast column at the level of the laminotomy; this was relieved by hyperflexion of the lumbar spine. With sustained hyperflexion of the lumbar spine, all neurologic deficits were completely resolved within 5 days. Lumbar lordosis may be present when a patient lies in the supine position on a flat bed with the hips and knees extended; this may exacerbate dural constriction caused by an epidural clot following posterior lumbar spinal surgery. ( info) |
10/67. Changing curve pattern in infantile idiopathic scoliosis: family report with a follow-up of 15 years. STUDY DESIGN: The authors monitored a father and his six children, all of whom had idiopathic scoliosis for more than 13 years. OBJECTIVE: To analyze the similarities and differences in curve pattern between the family members. SUMMARY OF BACKGROUND DATA: The etiology and pathogenesis of idiopathic scoliosis are not well understood. Genetic factors play an important role, as is shown by the high concordance in monozygotic twins. methods: Radiographs from all family members were analyzed. Location and direction of the curve, Cobb angle, location of the apex, and rib-vertebra angle difference were compared. RESULTS: A father and his six children had idiopathic scoliosis. The curves differed within the family and changed during follow-up. The father had a left convex thoracic scoliosis of 42 degrees. Only three of the children had a curve of similar severity, and only four children had a left convex thoracic scoliosis. In three children the curve pattern changed during follow-up. CONCLUSIONS: This report of idiopathic scoliosis in a father and six children supports the present theories on a genetic origin of the disorder but also stresses the importance of dynamic processes in the development of scoliosis. ( info) |