Spinal surgery for a late-onset axial myopathy.

Axial myopathies with paraspinal predominance usually present with dropped head, abnormal posture or rigidity of the spine. Management of axial myopathy can be difficult and there is little data in the literature about surgical treatment. We discuss a case of axial myopathy with late-onset scoliosis and dropped head, focusing on the surgical management of the case.

Two-stage anterior and posterior fusion versus one-stage posterior fusion in patients with Scheuermann's kyphosis.

AIMS: Whether a combined anteroposterior fusion or a posterior-only fusion is more effective in the management of patients with Scheuermann's kyphosis remains controversial. The aim of this study was to compare the radiological and clinical outcomes of these surgical approaches, and to evaluate the postoperative complications with the hypothesis that proximal junctional kyphosis would be more common in one-stage posterior-only fusion. METHODS: A retrospective review of patients treated surgically for Scheuermann's kyphosis between 2006 and 2014 was performed. A total of 62 patients were identified, with 31 in each group. Parameters were compared to evaluate postoperative outcomes using chi-squared tests, independent-samples t-tests, and z-tests of proportions analyses where applicable. RESULTS: There were six postoperative infections in the two-stage anteroposterior group compared with three in the one-stage posterior-only group. A total of four patients in the anteroposterior group required revision surgery, compared with six in the posterior-only group. There was a significantly higher incidence of junctional failure associated with the one-stage posterior-only approach (12.9% vs 0%, p = 0.036). Proximal junction kyphosis (anteroposterior fusion (74.2%) vs posterior-only fusion (77.4%); p = 0.382) and distal junctional kyphosis (anteroposterior fusion (25.8%) vs posterior-only fusion (19.3%), p = 0.271) are common postoperative complications following both surgical approaches. CONCLUSION: A two-stage anteroposterior fusion was associated with a significantly greater correction of the kyphosis compared with a one-stage posterior-only fusion, with a reduced incidence of junctional failure (0 vs 3). There was a notably greater incidence of infection with two-stage anteroposterior fusion; however, all were medically managed. More patients in the posterior-only group required revision surgery. Cite this article: Bone Joint J 2020;102-B(10):1368-1374.

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.

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.

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

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.

A Preliminary Study to Assess Whether Spinal Fusion for Scoliosis Improves Carer-assessed Quality of Life for Children With GMFCS Level IV or V Cerebral Palsy.

BACKGROUND: Scoliosis affects 50% of children with Gross Motor Function Classification System (GMFCS) level IV or V cerebral palsy (CP). In children with complex neurodisability following intervention, the WHO considers quality of life (QoL) should be assessed to aid decision-making and assess the effects. This study assesses whether scoliosis surgery improves carer-assessed QoL for children with severe CP. METHODS: Retrospective review of 33 children (16 male:17 female) with GMFCS level IV/V CP and significant scoliosis. Fifteen underwent observational treatment during childhood, and 18 underwent surgery. Questionnaire and radiographic data were recorded over a 2-year period. The carer-completed Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire was used to assess QoL. RESULTS: In the observational group, Cobb angle and pelvic obliquity increased from 46 (40 to 60) and 8 degrees (0 to 28) to 62 (42 to 94) and 12 degrees (1 to 35). Mean CPCHILD score decreased from 50 (30 to 69) to 48 (27 to 69) (P<0.05). In the operative group, Cobb angle and pelvic obliquity decreased from 78 (52 to 125) and 14 degrees (1 to 35) to 44 (16 to 76) and 9 degrees (1 to 24). Mean CPCHILD score increased from 45 (20 to 60) to 58 (37 to 76) (P<0.05). Change in pain, and not presence of associated impairments, was the most significant factor affecting QoL changes for children in both groups. There was no difference in mobility, GMFCS level, feeding, or communication in either group before and after treatment. CONCLUSIONS: Nonoperative treatment for children with GMFCS level IV/V CP and a significant scoliosis was associated with a small decrease in carer-assessed QoL over 2 years. Spinal fusion was associated with an increase in QoL. Change in pain was the most significant factor affecting QoL changes, and is therefore an important factor to consider when deciding upon surgery. LEVEL OF EVIDENCE: Level III-therapeutic retrospective study.

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

Spinal 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 51 (40-90) and 10 (0-30) to 70 (43-111) and 14 (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 81 (50-131) and 14 (1-35) to 38 (10-76) and 9 (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.

Revertant fibres and dystrophin traces in Duchenne muscular dystrophy: implication for clinical trials.

Duchenne muscular dystrophy (DMD) is characterised by the absence of dystrophin in muscle biopsies, although residual dystrophin can be present, either as dystrophin-positive (revertant) fibres or traces. As restoration of dystrophin expression is the end point of clinical trials, such residual dystrophin is a key factor in recruitment of patients and may also confound the analysis of dystrophin restoration in treated patients, if, as previously observed in the mdx mouse, revertant fibres increase with age. In 62% of the diagnostic biopsies reports of 65 DMD patients studied, traces or revertants were recorded with no correlation between traces or revertants, the patients' performance, or corticosteroids response. In nine of these patients, there was no increase in traces or revertants in biopsies taken a mean of 8.23 years (5.8-10.4 years) after the original diagnostic biopsy. This information should help in the design and execution of clinical trials focused on dystrophin restoration strategies.

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.

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.

Scoliosis development in identical twins after intercostal thoracotomy for pulmonary artery sling correction.

We present a report on two monozygotic female twins who underwent a left-sided intercostal thoracotomy in the second month of life for pulmonary artery sling correction. Twenty-four years later, in adulthood, the identical twins had both developed right-sided thoracic scoliosis. No previous accounts of scoliosis development after intercostal thoracotomy for pulmonary artery sling correction have been reported.

Prevalence and clinical significance of superficial abdominal reflex abnormalities in idiopathic scoliosis.

To determine prevalence and significance of abnormal superficial abdominal reflexes (SARs) in idiopathic scoliosis. Study of 73 patients with presumed idiopathic scoliosis referred for magnetic resonance imaging (MRI), either as a routine pre-operative assessment (n=42) or because of abnormal symptoms or neurological signs (n=31). All patients were examined prior to magnetic resonance imaging (MRI), and the presence of abnormal SARs was noted. All patients then underwent MRI of the whole spine from the foramen magnum to the sacrum. The presence of Chiari 1 malformation and syrinx was recorded. The study group consisted of 11 males and 62 females with a mean age at time of MRI of 18 years (range 5-51 years) and a mean Cobb angle of 48 degrees (range 10-104 degrees). Abnormality of the SARs was recorded in eight cases (prevalence 11%). An abnormal MRI study was recorded in nine cases (12.3%), all patients having a syrinx and four having in addition, a Chiari 1 malformation. Of the patients with abnormal SARs, only 2 (25%) had an abnormal MRI study; 1 had unilateral absence of the reflexes whereas the other had complete absence of SARs. Of patients referred for MRI as a routine pre-operative assessment, 5 (11.6%) had an abnormal MRI study. In patients with idiopathic scoliosis, abnormality of the SARs was recorded in 11% of cases. Unilateral absence was present in one case only and was associated with the presence of syrinx. Other patterns of abnormality were not a useful indicator of underlying cord abnormality.

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.