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Study Design: A review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition., Objective: To acquaint orthopedic spine surgeons with identification patterns of ischiovertebral dysplasia in order to provide them with guidelines about spine management and which complications to expect., Summary of Background Data: Ischiovertebral dysplasia is a rare skeletal dysplasia that may appear in a sporadic fashion or be inherited with an autosomal dominant inheritance pattern. It is defined by the association of an ischiopubic ramus hypoplasia and a vertebral dysplasia. It leads to a specific spine deformity whose management and complications should be clarified., Methods: Thirty consecutive patients from 0 to 31 years of age with ischiovertebral dysplasia were included from 5 centers specialized in congenital spinal deformities. Frontal and sagittal Cobb angles before treatment, natural history of the curves, therapeutic options, and their complications were systematically analyzed., Results: All the patients had a vertebral dysplasia and 28 of them developed a spinal deformity. This deformity was an extremely severe thoracic kyphoscoliosis in 25 cases. The other deformities were a thoracolumbar scoliosis in 1 case and a thoracolumbar kyphosis in 2 cases. The management of the thoracic kyphoscoliosis was always challenging and complications included death by respiratory failure (3 cases) and neurological impairment (9 cases)., Conclusion: Recognizing the occurrence of ischioverterbral dysplasia is very important to allow for dedicated treatment. The authors advocate preoperative distraction and circumferential fusion to prevent progression of the curve and to avoid the potentially fatal sequelae associated with this disorder., Level of Evidence: 4.

Study Design: Outcome study to determine the internal consistency, reproducibility, and concurrent validity of the French-Canadian version of the Scoliosis Research Society 22 (SRS-22 fcv) patient questionnaire in France., Objective: To determine whether the SRS-22 fcv can be used in a population from France., Summary of Background Data: The SRS-22 has been translated and validated in multiple countries, notably in the French-Canadian language in Quebec, Canada. Use of SRS-22 fcv seems appropriate for evaluating adolescent idiopathic scoliosis in France. However, French-Canadian French is noticeably different from the French spoken in France, and no study has investigated the use of a French-Canadian version of a health-quality questionnaire in another French population., Methods: The methods used for validating the SRS-22 fcv in Quebec were adopted for use with a group of 200 adolescents with idiopathic scoliosis and 60 healthy adolescents in France. Reliability and reproducibility were measured by the Cronbach α and intraclass correlation coefficient (ICC), construct validity by factorial analysis, concurrent validity by the Short-Form of the survey, and discriminant validity by analysis of variance and multivariate linear regression., Results: In France, the SRS-22 fcv showed good global internal consistency (Cronbach α = 0.87, intraclass correlation coefficient = 0.92), a coherent factorial structure, and high correlation coefficients between the SRS-22 fcv and Short-Form of the survey (P < 0.001). However, reliability and validity were slightly less than that for the instrument's original validation and the validation of the SRS-22 fcv in Quebec. These differences could be explained by language and cultural differences., Conclusion: The SRS-22 fcv is relevant for use in France, but further development and validation of a specific French questionnaire remain necessary to improve the assessment of functional outcomes of adolescents with scoliosis in France., Level of Evidence: N/A.

Background: Surgical correction of coxa vara in children with osteopenic bone diseases could be very challenging. In this study, we describe a modified surgical technique for the correction of coxa vara in children with bone fragility. We also report the results and complications of this technique in 16 children (21 coxa vara) with osteopenic bone diseases., Methods: Charts, clinic notes, and radiology images of 16 patients (21 hips) who had a proximal femoral osteotomy for the treatment of coxa vara in osteogenesis imperfecta (18 hips) and fibrous dysplasia (3 hips) in our institution between 1996 and 2005 were reviewed. The modified surgery involved an intertrochanteric osteotomy and the use of Kirshner wires and intramedullary rods. Neck-shaft angle, Hilgenreiner-epiphyseal angle, and head-shaft angle were assessed at preoperative, postoperative, and final follow-up., Results: The average age at surgery was 8.3 years (range, 3.3-15.8 years). The average correction of the neck-shaft angle was from 84.6 to 114.4 degrees, and for the Hilgenreiner-epiphyseal angle, it was from 67.7 to 42 degrees at final follow-up. All osteotomies were healed at 3 months postoperatively. The mean follow-up was 4.29 years (range, 1.70-8.12 years). The average improvement in abduction and external rotation of the hips was 14 and 15 degrees, respectively. There were no cases of infection and 2 cases (12%) of implant-related complications. One patient with fibrous dysplasia needed replacement of the intramedullary rod and additional distal femoral osteotomy 1 week postsurgery. In another patient, the intramedullary rod had migrated proximally, which was corrected 5 months postsurgery by advancing the rod distally., Conclusion: Results suggest that this surgical technique provides satisfactory correction of coxa vara in children with osteopenic bone diseases., Clinical Relevance: The described surgical technique used to correct coxa vara is reproducible and safe and has few complications. Furthermore, the size of the implants used allows surgery to be performed in very young children.

Study Design: Retrospective case series of 16 consecutive patients., Objective: To evaluate the results of scoliosis surgery in Prader-Willi syndrome (PWS) with special emphasis on the complications and their risk factors., Summary of Background Data: PWS is a rare genetic disorder characterized by obesity, hypotonia, and frequent scoliosis. The literature dealing with scoliosis surgery in PWS consists of only few case reports. Surgical treatment was reported to be difficult with frequent and severe complications., Methods: Sixteen patients (3 males, 13 females) in 63 presenting scoliosis and PWS were operated on between 1974 and 2004. Preoperative, postoperative and last follow-up clinical and radiologic data were analyzed. Complications, treatment, and outcome were investigated., Results: Mean age at scoliosis diagnosis was 6.2 years (range 0.5-13.5). Mean age at surgery was 12.3 years (range 5-15). Mean follow-up was 5.4 years (range 2-18). There were 9 major complications (4 severe kyphosis above fusion, 2 deep infections, 1 transient paraplegia, 1 pseudarthrosis, 1 delayed wound healing). The 4 kyphosis required reoperation, 3 of which were complicated with permanent spinal cord injury. Minor complications affected 6 patients., Conclusion: Scoliosis surgery is frequently necessary in PWS and is associated with high rate of complications. These are often related to specific features of this syndrome the surgeon should recognize and consider.

Study Design: We have retrospectively reviewed 11 chronic hemodialysis patients with cervical destructive spondyloarthropathy responsible for neural impairment 1 year after surgery and at last follow-up., Objective: To evaluate clinical and radiologic outcomes, and necessity of vertebral block excision., Summary of Background Data: Destructive spondyloarthropathy of the cervical spine is associated with long-term hemodialysis for chronic kidney failure. Spinal cord compression and neurologic troubles occur in a few cases. Surgical treatment remains controversial because these are debilitated patients with multiple organ failures., Methods: All 11 patients had unstable cervical spondylolisthesis, and 10 had kyphotic vertebral fusion involving at least 2 vertebrae. We performed interbody bone grafting (cement in 1 case) and stabilized with a plate. In 6 of the 10 patients with vertebral block, excision of the block was performed., Results: No patients were lost to follow-up. One patient died 2 days after the operation. There were 2 other patients who required early surgical revision (i.e., a corporectomy followed by early graft expulsion). Bone healing settled in all patients. One year after surgery, patients had almost complete resolution of the pain and satisfactory neurologic recovery. Improvement was evaluated according to the Nurick classification., Conclusion: Functional and neurologic results were similar whether the patients did or did not undergo vertebral block excision, suggesting that stabilizing the unstable level may be sufficient in patients with neurologic impairment. Excision of spontaneous vertebral blocks should be avoided to minimize the morbidity of surgery in these debilitated patients with a limited life expectancy.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The hip is usually affected later, and its involvement is the most common cause of disability in JIA. Failure of medical and preventive treatment, significant joint destruction, or multiarticular involvement may necessitate total hip arthroplasty (THA) to restore good function. Related clinical trials show initially good THA results in most of the series, but long-term outcomes are uncertain due to a significant arthroplasty loosening rate in the initial years, particularly with cemented stems. The authors report the results of 62 noncemented THAs in 34 children with JIA after an average follow-up of 6 years (range 3-13). Mean age at surgery was 18.3 years (range 11.8-31) and 14 of the 34 children had active disease. Clinical results were good for hip function but less for global function. There were no infections. Two acetabular cups early in the series failed due to poor primary fixation and had to be revised. Survivorship analysis was performed with the Kaplan-Meier method. At 13 years, the survival rate was 100% for the femoral component and 90.1% for the acetabular component.

Traumatic dislocation of the hip in childhood is uncommon and can be a consequence of minor trauma. The authors report a series of 35 dislocations in skeletally immature patients. Most were isolated posterior dislocations without acetabular lesions. In 75% of cases, reduction of the dislocation was easy. Nine children required surgery to remove interposed joint capsule and/or osteochondral fragments to achieve anatomic reduction. Outcomes were generally good, except in one patient in whom a displaced fracture of the femoral physis was followed by total head avascular necrosis. One case of partial necrosis had a satisfactory outcome. Epiphyseal necrosis, though uncommon, appeared to be inconsistent to prevent and hard to predict. Bone scan seems to be more effective than MRI for the detection of necrosis.

Fractures of the odontoid process are rare in children. We retrospectively reviewed 15 cases in children younger than 6 years, with an average follow-up of 4 years and 3 months. Eight of these patients had neurologic involvement; magnetic resonance imaging (MRI) changes were seen in the spinal cord at the cervicothoracic junction in six. This neurologic injury was thought to be due to the major anterior displacement of the upper spine, causing spinal cord stretch at the spinal apex of the cervical and thoracic spine. Eight patients secured in forward-facing car seats were injured as the result of motor vehicle accident. Conservatively treated fractures fused without problem. By contrast, the three cases operated on as the initial management of the fracture had complications postoperatively.