Jan Lehovsky

Management of scoliosis in Duchenne muscular dystrophy : a large 10-year retrospective study

Scoliosis affects 75 to 90% of patients with non-ambulant Duchenne muscular dystrophy (DMD). Spinal surgery is the treatment of choice but the indication varies among centres. Some offer surgery to all non-ambulant patients, irrespective of scoliosis severity. Early surgery has the advantage of targeting DMD when cardiorespiratory function is preserved, but not all patients develop scoliosis. We report our 10-year experience of scoliosis management in 123 patients with DMD who were at least 17 years old at the time of the study. Scoliosis was absent in 10%, and mild, non-progressive (at least 30 degrees ) in 13% of patients. Another 13% had moderate scoliosis (31-50 degrees ) and were managed conservatively. Surgery was considered in 57% (70/123) of patients with scoliosis greater than 50 degrees and eventually performed in 35%. The remaining patients either refused surgery (9%) or were unfit because of cardiorespiratory compromise (13%). In a further 7%, scoliosis (greater than 50 degrees ), first noted after 14 years of age, was progressing slowly and surgery was not performed. At 17 years there was no difference in survival, respiratory impairment, or sitting comfort among patients managed conservatively or with surgery. One-third (44/123) of our patients were managed satisfactorily without receiving spinal surgery. We provide insight into the natural history of scoliosis in DMD that should help families and clinicians with decision-making when surgery is considered.

Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity

Traditionally, spinal fusion has been denied to patients with scoliosis secondary to Duchennes muscular dystrophy (DMD) when their forced vital capacity (FVC) is less than 30–40% of predicted values (PFVC). The reasons for this decision are a theoretically increased risk of adverse events from a prolonged anaesthetic and extensive surgery. This paper presents a retrospective analysis of 30 patients with DMD scoliosis who underwent posterior spinal fusion at the Royal National Orthopaedic Hospital. Two subgroups of patients were compared: those with more than 30% PFVC (17 patients) and those with less than 30% PVFC (13 patients). One patient in each group required a temporary tracheotomy and there were nine complications in total. The post-operative stay for patients in each group was similar (24 days in the >30% group, 20 days in the <30% group) and the complication rate was comparable with other published series. We conclude that spinal fusion can be offered to patients with DMD even in the presence of a low FVC.

Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina.

Study Design. Case report. Objectives. To report a rare case of radiation-induced malignant peripheral nerve sheath tumor of the cauda equina 10 years after treatment for testicular seminoma. Summary of Background Data. Development of malignant peripheral nerve sheath tumor after irradiation is well recognized and often associated with a dismal prognosis. There have been isolated reports of malignant peripheral nerve sheath tumor developing in sites of previous irradiation for testicular seminoma. Methods. Retrospective review of case records and imaging. Results. A 38-year-old man presented with signs of cauda equina syndrome. Ten years previously, he had undergone right radical inguinal orchidectomy and adjuvant para-aortic radiotherapy as treatment for Stage I testicular seminoma. Magnetic resonance imaging demonstrated an inoperable intra- and extradural tumor leading to significant cauda equina compression. CT-guided biopsy revealed a diagnosis of malignant peripheral nerve sheath tumor, most likely due to previous radiotherapy. His clinical condition did not improve, and he underwent a course of palliative chemotherapy. Conclusions. Postirradiation malignant peripheral nerve sheath tumors are rare and occur in a population at high risk of developing second malignancies. The authors report the fourth case resulting from adjuvant radiotherapy for testicular seminoma, with the present report being the first report of extensive intradural tumor leading to cauda equina syndrome.

Development of lumbar high intensity zone on axial loaded magnetic resonance imaging.

Study Design. Case report. Objectives. To report the development of high intensity zones in the lumbar posterior anulus on axial loaded magnetic resonance imaging. Summary of Background Data. The high intensity zone is seen in the posterior anulus of degenerate lumbar discs on T2-weighted magnetic resonance images and represents a radial anular tear. Although the specificity of the high intensity zone for a concordantly painful anular tear in patients undergoing discography for discogenic low back pain is high, the sensitivity of magnetic resonance imaging for identification of discographically demonstrated anular tears is approximately 30%. Methods. A 55-year-old female with chronic low back pain was imaged with lumbar spine magnetic resonance imaging before and following axial loading with an magnetic resonance imaging-compatible compression device. Results. The nonloaded study demonstrated degeneration of the lower four lumbar discs. Following axial loading, posterior central high intensity zones were identified at the L2–L3 and L4–L5 levels. Conclusion. High intensity zones developed on axial loaded magnetic resonance imaging of the lumbar spine. We postulate that lumbar spine magnetic resonance imaging with axial loading may increase the sensitivity of magnetic resonance imaging for the detection of high intensity zones.

Osteoblastoma crossing the sacro-iliac joint

Osteoblastoma is a rare primary bone tumour which is well-described in the spine and sacrum. We report a case of histologically confirmed osteoblastoma in a 20-year-old male which crossed the sacroiliac joint. This has not previously been described to our knowledge. The imaging features of osteoblastoma and sacroiliac joint involvement by bone tumours are reviewed.

Does Spinal Fusion and Scoliosis Correction Improve Activity and Participation for Children With GMFCS level 4 and 5 Cerebral Palsy

AbstractSpinal fusion is used to treat scoliosis in children with cerebral palsy (CP). Following intervention, the WHO considers activity and participation should be assessed to guide intervention and assess the effects. This study assesses whether spinal fusion for scoliosis improves activity and participation for children with severe CP.Retrospective cohort study of 70 children (39M:31F) with GMFCS level 4/5 CP and significant scoliosis. Thirty-six underwent observational and/or brace treatment as the sole treatment for their scoliosis, and 34 underwent surgery. Children in the operative group were older and had worse scoliosis than those in the observational group. Questionnaire and radiographic data were recorded over a 2-year period. The ASKp was used to measure activity and participation.In the observational group, Cobb angle and pelvic obliquity increased from 51o (40–90) and 10o (0–30) to 70o (43–111) and 14o (0–37). Mean ASKp decreased from 16.3 (1–38) to 14.2 (1–36). In the operative group, Cobb angle and pelvic obliquity decreased from 81o (50–131) and 14o (1–35) to 38o (10–76) and 9o (0–24). Mean ASKp increased from 10.5 (0–29) to 15.9 (3–38). Spinal-related pain correlated most with change in activity and participation in both groups. There was no difference in mobility, GMFCS level, feeding or communication in either group before and after treatment.In children with significant scoliosis and CP classified within GMFCS levels 4 and 5, spinal fusion was associated with an improvement in activity and participation, whereas nonoperative treatment was associated with a small reduction. Pain should be carefully assessed to guide intervention.

Metastatic pulmonary intimal sarcoma presenting as cauda equina syndrome: first report of a case.

Study Design. Case report and clinical discussion. Objective. To describe a rare case of metastatic pulmonary intimal sarcoma presenting as cauda equine syndrome. Summary of Background Data. Pulmonary artery sarcoma is a rare tumor, frequently misdiagnosed as pulmonary embolism, and although it is highly aggressive, metastasis to bone is very rare. In our case, the tumor metastasised to the vertebral column, which lead the patient to present with cauda equine-type symptoms. Methods. We report the clinical and imaging features in a 38-year-old female presenting with back pain and weakness of the legs. Although initially misdiagnosed as a primary bone tumor of the vertebral column and a concurrent pulmonary embolism, the final diagnosis of pulmonary artery sarcoma with spinal metastasis, presenting as cauda equina syndrome was made. Results. At surgery, the mass was excised with the pulmonary valve and replaced with a homograft. The histology demonstrated an undifferentiated intimal sarcoma of the pulmonary artery. When stable enough the patient underwent a posterior L4-S1 decompression laminectomy after which she regained the power in the legs. The histology also showed a metastatic undifferentiated intimal sarcoma likely to have come from the pulmonary artery. The patient went on to receive a more formal vertebrectomy, front and back repair, and radiotherapy. Her 1-year follow-up computed tomography (CT) chest showed evidence of further metastases. Conclusion. To our knowledge, this is the first case of metastatic pulmonary intimal sarcoma presenting as cauda equine syndrome described in the literature.

A retrospective review to assess whether spinal fusion and scoliosis correction improved activity and participation for children with Angelman syndrome.

Abstract Objective: This study investigates outcome of scoliosis treatment for 11 children with Angelman syndrome (AS), with particular focus on activity, participation and the musculoskeletal factors that may affect these outcomes. Methods: Retrospective review of medical records, radiographs and questionnaires administered to caregivers of 11 children (8M:3F) with AS and scoliosis. Six underwent observational treatment during childhood and five underwent spinal fusion. The Activities Scale for Kids (ASKp) questionnaire was used to measure activity and participation. Questionnaire and radiographic data were recorded over a 2 year period. Results: In the observational group, scoliosis increased from 31° to 46°. Mean ASKp decreased from 13.8 to 11.9 (p = 0.06). In the operative group, scoliosis decreased from 68° to 29°. Mean ASKp increased from 11.4 to 15.9 (p < 0.01). There was also a reduction in spinal-related pain and mean number of hospital admissions for chest infection. However, there was a 60% major complication rate. There was no difference in mobility, GMFCS level, feeding or communication in either group before or after treatment. Conclusion: In children with significant scoliosis and AS, spinal fusion was associated with a small improvement in activity and participation, reduction in pain and a decrease in frequency of severe chest infections. Non-operative treatment resulted in progression of scoliosis during childhood and decrease in activity.

Risk of Neurological Injuries in Spinal Deformity Surgery.

Study Design. A retrospective study. Objective. Rate of neurological injuries is widely reported for spinal deformity surgery. However, few have included the influence of the subtypes and severity of the deformity, or anterior versus posterior corrections. The purpose of this study is to quantify these risks. Summary of Background Data. The risk of neurological injuries was examined in a single institution. Quantification of risk was made between operations, and for different subtypes of spinal deformity. Methods. Prospectively entered neuromonitoring database between 2006 and 2012 was interrogated, including all deformity cases under 21 years of age. Tumor, fracture, infection, and revision cases were excluded. All major changes in monitoring (“red alerts”) were identified and detailed examinations of the neuromonitoring records, clinical notes, and radiographs were made. Diagnosis, deformity severity, and operative details were recorded. Results. Of 2291 deformity operations, there were 2068 scoliosis (1636 idiopathic, 204 neuromuscular, 216 syndromic, 12 others), 89 kyphosis, 54 growing rod procedures, and 80 operations for hemivertebra. Six hundred ninety-six anterior and 1363 posterior operations were performed for scoliosis (nine not recorded), and 38 anterior and 51 posterior kyphosis corrections. Sixty-seven “red alerts” were identified (62 posterior, five anterior). Average Cobb angle was 88°. There were 14 transient and six permanent neurological injuries. One permanent injury was sustained during kyphosis correction and five during scoliosis correction. Common surgeon reactions after “red alerts” were surgical pause with anesthetic interventions (n = 39) and the Stagnara wake-up test (n = 22). Metalwork was partially removed in 20, revised in 12, and completely removed in nine. Thirteen procedures were abandoned. Conclusion. The overall risk of permanent neurological injury was 0.2%. The highest risk groups were posterior corrections for kyphosis, and scoliosis associated with a syndrome. Four percent of all posterior deformity corrections had “red alerts,” and 0.3% resulted in permanent injuries compared with 0.6% “red alerts” and 0.3% permanent injuries for anterior surgery. The overall risk for idiopathic scoliosis was 0.06%. Level of Evidence: 3

Do Growing Rods for Idiopathic Early Onset Scoliosis Improve Activity and Participation for Children

Objective To investigate whether growing rod surgery for children with progressive idiopathic early onset scoliosis (EOS) effects activity and participation, and investigate factors that may affect this. Study design Multicenter retrospective cohort study using prospectively collected data on 60 children with idiopathic EOS and significant scoliosis (defined as a Cobb angle >40°). Thirty underwent brace treatment, and 30, growth rod surgery. Questionnaire and radiographic data were recorded at 1 year. The validated Activities Scale for Kids performance version (ASKp) questionnaire was used to measure activity and participation. Results In the brace group, Cobb angle increased from 60° to 68°. There was no change in ASKp score. In the operative group, Cobb angle decreased from 67° to 45°. ASKp decreased from 91 to 88 (P < .01). Presence of spinal pain correlated with greater reduction in activity and participation scores in both groups, as did occurrence of complications in the operative group (P < .05). Both treatments permitted growth of the immature spine. Conclusions In children with significant idiopathic EOS (Cobb angle>40°), growth rod surgery was associated with a reduction in activity and participation and Cobb angle, whereas brace treatment was associated with an increase in Cobb angle and no change in activity and participation. Pain was the most important factor affecting activity and participation in both groups.