51/868. Fusion of spine in children scoliosis with frozen & radiation--sterilized bone allograft. 102 children have been treated at the Institute of traumatology, Orthopaedics & neurosurgery a result of scoliosis. In all of these multi-step treatment has been applied. Initially a telescopic rod was implanted into the spine. allografts were introduced around the rod hook, after 6 or 8 months, when angle of scoliosis increased, the rod was exchanged for a longer one. The final step was performed when conditions permitted and the scoliosis was markedly corrected and the fusion of the spine with the solid allograft was accomplished. The result of treatment was evaluated 1 to 7 years after surgery. Bone allografts were rebuilt within 6 month. Correction of scoliosis of 50% to 70% was achieved in all cases. ( info) |
| The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found. ( info) |
53/868. Multicore myopathy: respiratory failure and paraspinal muscle contractures are important complications. Three ambulant males with multicore myopathy, a rare congenital myopathy, are reported with nocturnal hypoventilation progressing to respiratory failure at the age of 9, 13, and 21 years. Deterioration in these individuals occurred over several months without any precipitating event. patients had clinical evidence of nocturnal hypoventilation with hypoxaemia and hypercapnia. Forced vital capacity was significantly reduced (20 to 43% of predicted level). These parameters improved on institution of overnight ventilation using a BiPAP pressure support ventilator with face mask or nasal pillows with O2 saturation maintained above 90% overnight and an increase in forced vital capacity by as much as 100% (0.3 to 0.6 litres). This was matched by a symptomatic and functional improvement. Also present in these patients and not previously reported is the association of multicore myopathy with paraspinal contractures which produce a characteristic scoliosis described as a 'side-sliding' spine. This may be improved by spinal bracing or surgery. ( info) |
54/868. Unilateral absence of the clavicle with rapidly progressive scoliosis in an 8-year-old. We report a possible association of unilateral absence of a clavicle with rapidly progressive scoliosis. cleidocranial dysplasia (CCD) is an autosomal dominant disorder that is characterized by defective bone formation. The clavicle, pelvis, and skull are the most commonly affected bones. A review of the literature found two cases of CCD and scoliosis. Unilateral absence of the clavicle in association with rapidly progressing scoliosis has not been previously reported. review of the patient's charts and radiographs from age 8 to 17 years, 5 years after treatment with posterior spinal instrumentation is presented, together with a review of the literature. Our patient initially presented without any spinal deformity until age 9, when she had a 10 degree curve between C-8 and L-T. Eighteen months later, the curve progressed to 52 degrees, Risser 1. Associated anomalies include posterior-element hypoplasia of the thoracic spine and posterior fusion of C4-6. She was treated with posterior spinal instrumentation from C-8 to L-4 without complications. Correction was maintained at 5-year follow-up. There may be an association between unilateral absence of the clavicle and rapid progression of scoliosis in immature children. We hypothesize that the asymmetrical influence of the unilateral absent clavicle may have played a causative role in her rapidly progressive scoliosis. ( info) |
55/868. D-2-hydroxyglutaric aciduria in association with spondyloenchondromatosis. D-2-hydroxyglutaric aciduria is a rare metabolic disorder, first reported in 1980, and does not yet have a clinically specific presentation pattern nor any specific treatment regime. We report a girl born with this uncommon metabolic disorder, who, at the age of 12 months, was also found to have a severe crippling form of skeletal dysplasia, spondyloenchondromatosis. ( info) |
56/868. Stress fracture of the hip and pubic rami after fusion to the sacrum in an adult with scoliosis: a case report. Correction of adult scoliosis frequently involves long segmental fusions, but controversy still exists whether these fusions should include the sacrum. It has been suggested that forces associated with activities of daily living transfer the stresses to the remaining levels of the spine and to the pelvis. The case described here was a 43-year-old woman with scoliosis and chronic back pain refractory to non-surgical modalities. Radiographically, the patient had a 110 degree lumbar curve. An anterior and posterior fusion with Luque-Galveston instrumentation was performed. Six months postoperatively the patient returned with a 2-week history of right hip pain with no history of trauma. There was radiographic evidence of a displaced femoral neck fracture and pubic rami fractures. The femoral neck fracture was treated with a total hip replacement. Further surgeries were required to correct a lumbar pseudoarthrosis and hardware failure. We believe that this case provides evidence that fusion into the lumbosacral junction may distribute forces through the pelvic bones and hip resulting in stress and potential hardware complications, especially in patients at risk due to osteopenic conditions. ( info) |
57/868. Thoracolumbar kyphoscoliosis in Larsen's syndrome. A case report. Severe thoracolumbar kyphosis in Larsen's syndrome is rare. A successful 10-year treatment course, including T2-L4 subfascial rod placement when the patient was 6 years 3 months of age, nine periodic lengthenings, and definitive surgery at age 14 years 4 months is presented. From preoperative to 2-year postoperative definitive surgery, the patient's T1 to S1 spine length increased from 19 to 36 cm. The patient's 83 degrees thoracolumbar kyphosis was transformed to 45 degrees kyphosis and 28 degrees lumbar lordosis, and scolioses of 95 degrees and 65 degrees were reduced to 64 degrees and 50 degrees. ( info) |
58/868. A prospective comparison of neurogenic mixed evoked potential stimulation methods: utility of epidural elicitation during posterior spinal surgery. STUDY DESIGN: A prospective study of 50 patients who underwent posterior thoracic or thoracolumbar instrumented spinal surgery from June 1998 through June 1999. OBJECTIVES: To highlight the advantages and disadvantages of neurogenic mixed evoked potential (NMEP) data obtained with three methods of stimulation: epidural, spinous process, and percutaneous. SUMMARY OF BACKGROUND DATA: Reports in the literature have established the efficacy of epidural, spinous process, and percutaneous stimulation of the NMEP response. The three methods have not been compared for reliability, sensitivity, and specificity. methods: The study group consisted of 50 patients who underwent posterior thoracic or thoracolumbar instrumented spinal surgery from June 1998 through June 1999. Somatosensory-evoked potentials were used to monitor upper and lower extremities. An attempt to obtain NMEPs was made in all patients by using percutaneous (PERC-NMEP), spinous process (SP-NMEP), and epidural (EPI-NMEP) stimulation. These data were evaluated for reliability, sensitivity, and specificity. The number of minutes monitored in the postcorrection period were calculated for each method, and stimulus intensities were noted. RESULTS: In the current study, PERC-NMEPs were obtained in 88% of the patients and were maintained in 91% of those cases, SP-NMEPs were obtained in 96% and maintained in 77%, and EPI-NMEPs were obtained in 100% and maintained in 88%. data collection continued in the postcorrection period for 46 minutes for PERC-NMEPs, 19 minutes for SP-NMEPs, and 23 minutes for EPI-NMEPs. The study group had no true-positive or false-negative findings. CONCLUSIONS: Results showed that EPI-NMEPs provide reliable data in a greater number of patients than either SP-NMEPs or PERC-NMEPs. However, PERC-NMEP data are readily maintained during and after the critical time window after corrective spinal maneuvers. The NMEPs elicited with both percutaneous and epidural stimulation have a useful role in an intraoperative spinal cord-monitoring protocol. ( info) |
59/868. Primary tethered cord syndrome: diagnosis and treatment of an insidious defect. Failure to recognize the signs and symptoms of tethered cord syndrome in patients with spina bifida occulta can result in tragic consequences. Of patients with tethered spinal cord, 35% have bowel and/or bladder dysfunction. scoliosis, foot or leg length discrepancies, pes cavus, and varus or valgus deformities also can occur. Early assessment and intervention by the neuroscience nurse who is familiar with these clinical signs can help prevent significant deformities and irreversible neurological deficits. ( info) |
60/868. Intragastric migration of a pacemaker implanted in the pleural space. We have encountered a 12-year-old boy with a pacemaker implanted in the left pleural space during early infancy that migrated into the stomach through the diaphragm due to severe scoliosis and occult infection. The intrathoracic implantation of a pacemaker in an infant should be assessed more carefully, taking the volume of the unit into consideration, because of this potentially life-threatening complication. ( info) |