Further delineation of spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type, with emphasis on diagnostic features.

Further delineation of a generalized bone dysplasia which we call spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type is presented. This dwarfing condition has several serious complications, with the most common cause of death being spinal cord damage secondary to atlantoaxial instability. It is a heritable condition with an autosomal recessive mode of transmission. Radiologic diagnostic criteria are developed on the basis of studies in 8 patients with the oldest being between 4 and 5 years old. The condition is clinically and radiographically apparent neonatally or in early infancy, and it is probable that all or almost all affected individuals will come to medical attention in the age range screened by this study.

The value of computed tomography in thoracolumbar fractures. An analysis of one hundred consecutive cases and a new classification.

We studied 100 consecutive patients with potentially unstable fractures and fracture-dislocations by multiplane computed tomography. The mechanism of failure of the middle osteoligamentous complex of the spine (posterior longitudinal ligament, posterior part of the vertebral body, and posterior annulus fibrosus) was determined by three-dimensional analysis. Three modes of middle-column failure were used to classify the injuries: axial compression (seventy-three patients), axial distraction (fifteen patients), and translation within the transverse plane (twelve patients). Fifty of eighty-six patients who were evaluated in the acute phase of injury underwent operative stabilization, and the mechanism of middle-column disruption determined the type of instrumentation that was used. Compression and distraction injuries of the middle complex could be appropriately treated by Harrington distraction and compression instrumentation, respectively. However, in translational injuries (torn posterior longitudinal ligament) routine Harrington instrumentation was contraindicated due to the risk of overdistraction. Translational injuries were associated with the greatest degree of instability and often had complete ligament discontinuity at the level of the affected vertebrae. Patients with a translational injury had the most severe neural deficits (six of eleven patients studied acutely having a complete spinal cord lesion). Translational injuries of the middle column were treated by segmental spinal instrumentation to provide strong fixation with minimum risk of neural sequelae from passing sublaminar wires. Moreover, postoperative use of a cast over insensate skin was not required. Computed tomography was more sensitive than any other modality in the diagnosis of disruption of the posterior elements in unstable burst fractures, and computer-reconstructed sagittal images were accurate in evaluating the nature of facet-joint failure in distraction injuries. Computed tomography with metrizamide proved superior to either conventional tomography or myelography alone in localizing the site of neural canal compromise in acute thoracolumbar injuries. The mode of failure of the middle osteoligamentous complex as visualized by computed tomography determined the pattern of spinal injury, the severity of the neural deficit, the degree of instability, and the type of instrumentation required.

The natural history of spondylolysis and spondylolisthesis.

We performed a prospective roentgenographic study to determine the incidence of spondylolysis, spondylolisthesis, or both, in 500 unselected first-grade children from 1955 through 1957. The families of the children with spondylolysis were followed in a similar manner. The incidence of spondylolysis at the age of six years was 4.4 per cent and increased to 6 per cent in adulthood. The degree of spondylolisthesis was as much as 28 per cent, and progression of the olisthesis was unusual. The data support the hypothesis that the spondylolytic defect is the result of a defect in the cartilaginous anlage of a vertebra. There is a hereditary pre-disposition to the defect and a strong association with spina bifida occulta. Progression of a slip was unlikely after adolescence and the slip was never symptomatic in the population that we studied.

Neurologic and vascular complications of scoliosis surgery in patients with Ehlers-Danlos syndrome. A case report.

STUDY DESIGN: Case reports. OBJECTIVES: Description of neurologic and vascular complications of scoliosis surgery to children and adolescents with ehlers-danios syndrome. SUMMARY OF BACKGROUND DATA: There have been no previous reports of neurologic and vascular complications of scoliosis surgery in patients with Ehlers-Danlos syndrome. Scoliosis and vascular fragility are characteristics of certain types of Ehlers-Danlos syndrome. METHODS: Case reports and literature review. RESULTS: Subsequent to scoliosis surgery, three patients with Ehlers-Danlos syndrome developed neurologic complications: two developed paraplegia, and one developed unilateral foot and ankle weakness with transient neurogenic bladder. Another patient with Ehlers-Danlos syndrome experienced avulsion of segmental arteries during anterior spinal surgery. CONCLUSIONS: Patients with Ehlers-Danlos syndrome may be at high risk for neurologic and vascular complications consequent to scoliosis surgery, necessitating careful perioperative evaluation and management.

The halo-Milwaukee brace. Case series of a revived technique.

STUDY DESIGN: A case series in which the halo-Milwaukee brace was used for postoperative immobilization in children with complex congenital and developmental spinal deformities. OBJECTIVES: To describe the use of halo-Milwaukee orthosis in a pediatric population for stabilization of the cervical and upper thoracic spine. SUMMARY AND BACKGROUND DATA: Postoperative immobilization of the neck and upper thorax can be achieved with cervical orthoses, cervicothoracic lumbar orthosis, halo cast, Minerva jacket, or halo vest. In the young child or in individuals with severe deformities, prefabricated braces often do not provide adequate stability or predictable fit. The halo-Milwaukee brace has proven to be an effective and versatile technique in the management of complex pediatric spinal deformities. METHODS: Halo-Milwaukee brace immobilization was used in 12 patients after surgical stabilization of the upper thoracic or cervical spine. Technique and indications are discussed in this report. Surgical outcomes and complications were reviewed retrospectively in all cases. RESULTS: Application of the halo-Milwaukee brace was a clinically effective and safe means of controlling the upper thoracic and cervical spine. The orthosis was well tolerated and allowed access to the posterior incision. The brace is easily converted to a standard Milwaukee brace with neck ring. The pelvic segment of the brace is molded before surgery, and in most instances did not require postoperative modification. CONCLUSION: The halo-Milwaukee brace is a simple and convenient method of intraoperative and postoperative immobilization. The technique is applicable in patients who cannot be treated with more conventional off-the-shelf orthoses. The brace was well tolerated and allowed for early patient mobilization.

Somatosensory cortical evoked potential changes after deformity correction.

The somatosensory cortical evoked potentials recorded during posterior spine fusion and instrumentation for 99 consecutive patients with idiopathic scoliosis, 18 years of age or younger, were retrospectively reviewed. The potentials were recorded from scalp electrodes while synchronously stimulating both tibial nerves near the ankles. Signal changes observed during consecutive 30-minute time intervals after deformity correction were analyzed. No changes in neurologic status were observed postoperatively. Latency values tended to remain constant on average. A small, but statistically significant, decrease in the first two interpeak amplitudes was observed during the first 30-minute interval after deformity correction. The first interpeak amplitude recovered, while the second remained statistically significantly decreased. No patient had a decrease of greater than 50% in both of the first two amplitudes, which persisted throughout the 60-minute interval immediately after deformity correction. This study demonstrated a tendency for somatosensory cortical evoked potential interpeak amplitudes to decrease during the first 30 minutes after deformity correction. There was a great deal of individual variation, including amplitude increases in many patients. There was no evidence supporting an association between dramatic, sustained amplitude decreases and uncomplicated deformity correction. The value of thoroughly evaluating somatosensory cortical evoked potential signal trends while making intraoperative decisions is emphasized.

Variability of somatosensory cortical evoked potential monitoring during spinal surgery.

The intraoperative variability of somatosensory cortical evoked potentials (SCEPs) has been measured for 320 consecutive spinal surgeries and found to be a function of patient diagnosis, neuromuscular status, age, and procedural factors. In many cases, it is likely that this variability severely limits the reliability and usefulness of spinal cord monitoring in detecting early cord compromise. Patients with idiopathic scoliosis, spondylolisthesis, and pseudarthrosis have the smallest spontaneous variability and strongest amplitudes, while those with congenital, paralytic scoliosis, stenosis, or tumor have very variable, weak SCEPs. Patients with neurologic disorders, particularly cerebral palsy, myelomeningocele, Friedreich's ataxia, and peripheral deficits, also have high variability and weak amplitudes. A monitoring quality scoring system is proposed that may be useful during surgery in judging how well the SCEPs can discern surgically related changes in cord function from background variations.

Reduction of the intracanal fragment in experimental burst fractures.

An experimental investigation was carried out to create burst fractures and to evaluate the mechanisms and degree of reduction of the intracanal fragment with posterior instrumentation techniques in multisegmental human cadaver specimens. Reduction of the spinal fragment through kyphosis correction and distraction was evaluated using CT imaging. With kyphosis correction alone there was no decrease in canal compromise; in some cases there was a slight increase in canal compromise. Distraction, whether applied before or after kyphosis correction was the effective mechanism in reducing the fracture fragment. Kyphosis correction applied after distraction did not reduce the fragment further. Posterior devices that are used to treat burst fractures of the thoracolumbar spine with intracanal fragments should provide some form of distraction.

The Luque trolley revisited. Review of nine cases requiring revision.

This study is a retrospective review of nine patients who underwent Luque instrumentation without fusion from 1982-1984. Average age at surgery was 9 years. Average preoperative curve was 51 degrees (30 degrees-70 degrees). All nine patients have had at least one revision. All of the revisions were technically difficult secondary to extensive fibrosis and weakened laminar bone. Spontaneous fusion was documented in all nine patients, limiting further correction. Final follow-up curves averaged 51 degrees (25 degrees-90 degrees). Average gain in spinal height was 5.8 cm (2.3 in) but only a small portion was derived from the instrumented levels. Segmental spinal instrumentation without fusion in immature patients was not effective in control of spinal deformity, nor did it allow anticipated growth under the instrumented regions.

Spinal deformity in Charcot-Marie-Tooth disease.

STUDY DESIGN: This retrospective study reviewed 100 children with clinically and electrodiagnostically proven Charcot-Marie-Tooth disease (CMTD). OBJECTIVE: To determine the incidence of spinal deformity in children with CMTD. SUMMARY OF BACKGROUND DATA: A 10% incidence of kyphoscoliosis has been reported. METHODS: All charts and electrodiagnostic studies were reviewed to confirm that CMTD diagnostic criteria were met. Existing radiographs on 89 children were available. Cobb angles were measured and deformity was defined as scoliosis > or = 10 degrees and kyphosis > 40 degrees. RESULTS: Thirty-seven of 89 CMTD children had spinal deformity. There was scoliosis in 20, kyphoscoliosis in 14, and kyphosis in 3. In children with radiographs taken at maturity, 50% had deformity. The most common scoliotic pattern was a thoracic curve with convexity in either direction. Spinal deformity is more likely in female and Type I patients. CONCLUSIONS: This study found an incidence of 37%-50% spinal deformity in children with CMTD, with female and Type I patients at greatest risk. However, the deformity rarely required treatment.

Early clinical experience with the Syracuse I-Plate: an anterior spinal fixation device.

Sixteen patients were treated with a new anterior internal fixation device after thoracolumbar or lumbar decompression, and fusion with bone grafting. Ten patients had acute burst fractures, four had metastatic tumors, and two had old, healed fractures with deformity. In the acute fracture group, eight patients had neurologic deficits and seven patients experienced improvement. Six patients had lesions of the conus medullaris, all of which improved. The four patients with metastatic tumors underwent surgery for back and leg pain and all gained significant relief. Two patients had correction of old fracture deformity with satisfactory outcome. Complications were minimal. The new anterior stabilization device provided early stability, allowed early patient mobilization, was easy to insert, and has a low profile. Late collapse, non-union, and kyphotic deformity have not been noted thusfar.

Lower extremity compartment syndrome in an adolescent with spinal cord injury.

OBJECTIVE: Describe the unusual complication of lower extremity compartment syndrome occurring in an adolescent with spinal cord injury (SCI). METHODS: Case presentation. RESULTS: A 17-year-old male with C5 ASIA A complete SCI developed a compartment syndrome of his lower leg on the ninth day postinjury. Presenting signs included an equinus deformity of the foot, blackened induration over the anterior tibia, circumferential erythematous markings over the calf, large urticarial lesions over the knee, and calf swelling. The presumed etiology of the compartment syndrome was excessive pressure from elastic wraps, which were placed over gradient elastic stockings. Pressures were 51 mmHg in the superficial posterior, 50 mmHg in the deep posterior, 33 mmHg in the anterior, and 34 mmHg in the peroneal compartments. The patient also developed rhabdomyolysis with myoglobinuria. In addition to supportive care, the patient underwent a dual incision fasciotomy for compartment release. CONCLUSIONS: The development of lower extremity compartment syndrome was probably a result of excessive pressure applied by elastic wraps. Elastic wraps should be used with caution in individuals with SCI.

Kyphectomy and segmental spinal instrumentation in young children with myelomeningocele kyphosis.

For children with myelomeningocele kyphosis, the ideal timing for spinal correction or the correct stabilization technique for preventing the recurrence of deformity are controversial issues. From 1988 to 1992, vertebral kyphectomy and long segment spinal fixation with a special lower-end contouring around the anterior aspect of the sacrum through the sacral foramina (the modified Gillespie's technique) were carried out on six young children with ages ranging from three years and four months to five years and 11 months (average, five years). The indications for surgery were recurrent skin breakdown and/or increasing functional deficits due to curve progression. A mean preoperative kyphosis of 126.3 degrees was reduced to 21.8 degrees postoperatively. There was one case of pseudarthrosis seen at the osteotomy site which was treated with anterior interbody grafting. With a mean follow-up period of 35 months (range, 10-48 months), all patients showed good maintenance of correction and no implant failure. The average loss of correction was 2.7 degrees. We believe that in these young, severely handicapped children, long segment fixation using the modified Gillespie's technique with full or partial segment fusion allows a quick resumption of upright activities, achieves satisfactory correction of kyphosis and minimizes the risk of recurrent deformity.

Chondrolysis and avascular necrosis: complications of slipped capital femoral epiphysis.

Chondrolysis and avascular necrosis (AVN) are serious complications after slipped capital femoral epiphysis (SCFE). Chondrolysis, or acute cartilage necrosis of the capital femoral epiphysis, can occur in treated and untreated SCFE. Its exact etiology has not been conclusively determined, and there may be some connection with hardware penetration into the joint and its development. Because chondrolysis can occur in both treated and untreated hips, an immune mechanism has been postulated. Although some evidence of immune reaction has been confirmed in the joint, no screening test has yet been developed to determine susceptible individuals. AVN is clearly related to impeded blood supply to the femoral head and to severely slipped unstable acute slips. Forceful reduction of the femoral head must be avoided. However, some slips are so unstable that reductions occur merely with positioning of the patient for operation. Improperly placed fixation devices may also play a role in the development of AVN. Chondrolysis and AVN may not be completely avoidable in the treatment of SCFE.

Cervical spine fusion: not protective of cervical spine injury and tetraplegia.

The occurrence of tetraplegic spinal cord injuries in two patients who had preexisting cervical spine fusions highlights the vulnerable nature of such patients. One patient sustained a C5-6 fracture-dislocation after a motor vehicle accident, despite a spontaneously fused cervical spine as a consequence of his juvenile rheumatoid arthritis. A second patient sustained a C3-4 distraction injury in a sporting injury 2 years after he had undergone a posterior cervical spine fusion because of an aneurysmal bone cyst. Prior to their spinal cord injuries, both patients had no apparent neurologic deficits, were relatively active, and followed no specific precautions for their spines.

Clubfoot deformity in Down's syndrome.

The association of Down's syndrome (trisomy 21) with clubfeet has not previously been elaborated. Eight patients with a total of 15 clubfeet were identified for review. Five of these had trisomy 21 noted by chromosomal analysis, and 1 had a mosaic pattern. Two patients did not have chromosomal documentation, but had characteristic features of Down's syndrome. Interestingly, 2 patients had evidence of arthrogryposis as well as Down's syndrome. Four of the 8 patients had other orthopedic anomalies, including scoliosis, atlantoaxial instability, brachydactyly, and coxa valga. All 8 patients had an initial period of casting prior to any surgical intervention. Fourteen of the 15 feet required surgical intervention to afford correction of the deformity. The 6 feet with relatively long-term follow up (average: 5 years) showed that there was 1 excellent, 4 good, and 1 fair result using the criteria of Turco. It appears that, even though Down's syndrome is usually characterized by ligamentous laxity, when clubfeet are associated with this syndrome they are often resistant to nonoperative treatment, and surgical treatment seems to produce an acceptable result.