Sean Molloy

A review of the surgical management of sacral chordoma

BACKGROUND Sacral chordomas are rare low-to-intermediate grade malignant tumours, which arise from remnants of the embryonic notochord. This review explores prognostic factors in the management of sacral chordomas and provides guidance on the optimal treatment regimens based on the current literature. PATIENTS AND METHODS Electronic searches were performed using MEDLINE, Embase and the Cochrane library to identify studies on prognostic factors in the management of sacral chordomas published between January 1970 and December 2013. The literature search and review process identified 100 articles that were included in the review article. This included both surgical and non-surgical studies on the management of sacral chordomas. RESULTS Sacrectomy with wide resection margins forms the mainstay of treatment but is associated with high risk of disease recurrence and reduced long-term survival. Adequate resection margins may require sacrifice of adjacent nerve roots, musculature and ligaments leading to functional compromise and mechanical instability. Large tumour size (greater than 5-10 cm in diameter), dedifferentiation and greater cephalad tumour extension are associated with increased risk of disease recurrence and reduced survival. Chordomas are poorly responsive to conventional radiotherapy and chemotherapy. CONCLUSION Operative resection with wide resection margins offers the best long-term prognosis. Inadequate resection margins, large tumour size, dedifferentiation, and greater cephalad chordoma extension are associated with poor oncological outcomes. Routine long-term follow-up is essential to enable early detection and treatment of recurrent disease.

Pisa syndrome in Parkinson's disease: a mobile or fixed deformity?

Background Although Pisa syndrome and scoliosis are sometimes used interchangeably to describe a laterally flexed postural deviation in Parkinsons disease (PD), the imaging findings of Pisa syndrome in PD have not been previously studied in detail. Methods Patients with PD and Pisa syndrome (lateral flexion >10° in the standing position) were examined clinically and underwent radiological assessment using standing radiograph and supine CT scan of the whole spine. Results Fifteen patients were included in this observational study. All patients had scoliosis on standing radiographs, and 12 had scoliosis persisting in the supine position. Scoliotic curves improved by a mean of 44% when patients moved from standing to supine. Only a quarter of patients with structural scoliosis had evidence of bony fusion on the side of their lateral deviation rendering their deformity fixed. Conclusions Pisa syndrome describes a patient who lists to the side whereas scoliosis is defined by spinal curvature and rotation and may not be associated with lateral flexion. The finding of ‘structural scoliosis’ in Pisa syndrome should not preclude intervening to improve posture as most patients had little or no evidence of structural bony changes even when the deformity had been present for a number of years.

Vertebral compression fractures – new clinical signs to aid diagnosis

INTRODUCTION Acute osteoporotic vertebral compression fractures are common and usually managed conservatively. However, a significant number will remain symptomatic, causing significant pain with considerable associated morbidity and mortality. These fractures can be effectively treated with cement augmentation. However, it is impossible to distinguish between an acute and a chronic healed fracture on plain radiographs. The definitive investigation is a magnetic resonance scan. The aim of this paper is to describe and evaluate two new clinical signs to help in the diagnosis of symptomatic fractures. A prospective study of 83 patients with suspected acute osteoporotic vertebral compression fractures was carried out. All patients had a full clinical assessment, which included closed-fist percussion of their spine and asking the patient to lie supine on the examination couch. All patients had a MRI scan. RESULTS The closed-fist percussion sign had a sensitivity of 87.5% and a specificity of 90%. The supine sign had a sensitivity of 81.25% and a specificity of 93.33%. CONCLUSIONS These tests will enable the practitioner to predict more accurately which patients have an acute fracture, guiding referral for further imaging.

Optimizing the management of patients with spinal myeloma disease

Myeloma is one of the most common malignancies that results in osteolytic lesions of the spine. Complications, including pathological fractures of the vertebrae and spinal cord compression, may cause severe pain, deformity and neurological sequelae. They may also have significant consequences for quality of life and prognosis for patients. For patients with known or newly diagnosed myeloma presenting with persistent back or radicular pain/weakness, early diagnosis of spinal myeloma disease is therefore essential to treat and prevent further deterioration. Magnetic resonance imaging is the initial imaging modality of choice for the evaluation of spinal disease. Treatment of the underlying malignancy with systemic chemotherapy together with supportive bisphosphonate treatment reduces further vertebral damage. Additional interventions such as cement augmentation, radiotherapy, or surgery are often necessary to prevent, treat and control spinal complications. However, optimal management is dependent on the individual nature of the spinal involvement and requires careful assessment and appropriate intervention throughout. This article reviews the treatment and management options for spinal myeloma disease and highlights the value of defined pathways to enable the proper management of patients affected by it.

Prognostic factors in the operative management of dedifferentiated sacral chordomas.

BACKGROUND Dedifferentiated chordomas are rare high-grade malignant spinal tumors for which there is minimal information to help guide treatment. OBJECTIVE To identify prognostic factors associated with increased risk of local recurrence, metastases, and reduced survival in a cohort of patients undergoing sacrectomy for de novo dedifferentiated sacral chordoma. METHODS Ten patients undergoing sacrectomy for histologically confirmed dedifferentiated chordoma at a specialist center were reviewed. There were 6 male and 4 female patients with a mean age of 66.7 years (range, 57-80 years) and mean follow-up of 36.7 months (range, 3-98 months). Data on prognostic factors were collected. RESULTS The commonest presenting symptom was lumbar/gluteal pain. Mean duration of preoperative symptoms was 3.6 months (range, 2-7 months). Local recurrence was seen in 7 patients; metastases occurred in 5 patients. After sacrectomy, 7 patients died at a mean of 41 months (range, 3-98 months). Tumor size >10 cm in diameter, amount of dedifferentiation within the conventional chordoma, sacroiliac joint infiltration, and inadequate resection margins were associated with increased risk of recurrence and reduced survival. Surgical approach, cephalad extent of primary tumor, and adjuvant radiotherapy did not affect oncological outcomes. CONCLUSION Dedifferentiated chordomas are aggressive malignant tumors with a higher risk of local recurrence, metastases, and early mortality than conventional chordomas. Tumor diameter >10 cm, marginal resection, and sacroiliac joint infiltration may be associated with increased risk of local recurrence and mortality. Those with a smaller burden of dedifferentiated disease (<1 cm) within the primary chordoma have a better prognosis. Patients should be counseled about these risks before surgery and should have regular follow-up for the detection of local recurrence and metastases.

A new extensile anterolateral retroperitoneal approach for lumbar interbody fusion from L1 to S1: a prospective series with clinical outcomes.

BACKGROUND CONTEXT A variety of surgical approaches have been used for cage insertion in lumbar interbody fusion surgery. The direct anterior approach requires mobilization of the great vessels to access the intervertebral disc spaces cranial to L5/S1. With the lateral retroperitoneal transpsoas approach, it is difficult to access the L4/L5 intervertebral disc space due to the lumbar plexus and iliac crest, and L5/S1 is inaccessible. We describe a new anterolateral retroperitoneal approach, which is safe and reproducible to access the disc spaces from L1 to S1 inclusive, obviating the need for a separate direct anterior approach to access L5/S1. PURPOSE This paper had the following objectives: first, to report a reproducible novel single-incision, muscle-splitting, anterolateral pre-psoas surgical approach to the lumbar spine from L1 to S1; second, to highlight the technical challenges of this approach and highlight approach-related complications; and third, to evaluate clinical outcomes using this surgical technique in a prospective series of L1 to S1 anterior lumbar interbody fusions (ALIFs) performed as part of a 360-degree fusion for adult spinal deformity correction. STUDY DESIGN This report used a prospective cohort study. PATIENT SAMPLE A prospective series of patients (n=64) having ALIF using porous tantalum cages as part of a two-stage complex spinal reconstruction from L1 to S1 were studied. OUTCOME MEASURES Data collected included blood loss, operative time, incision size, technical challenges, perioperative complications, and secondary procedures. Clinical outcome measures used included visual analogue scale (VAS) Back Pain, VAS Leg Pain, EuroQoL-5 Dimensions (EQ-5D), EQ-5D VAS, Oswestry Disability Index (ODI), and Scoliosis Research Society-22 (SRS-22). METHODS Pre- and postoperative radiographic parameters and clinical outcome measures were assessed. Mean follow-up time was 1.8 years. RESULTS Mean blood loss was 68±9.6 mL. The mean VAS Back Pain score improved from 7.5±1.25 preoperatively to 2.5±1.7 at 3 months (p=.02), 1.2±0.5 at 6 months (p=.01), and 1.4±0.6 at 1 year (p=.02). The mean ODI improved from 64.3±31.8 preoperatively to 16.6±14.7 at 3 months (p>.05), 10.7±6.0 at 6 months (p=.02), and 6.7±6.1 at 1 year (p=.01). There were no permanent neurologic, vascular, or visceral injuries. One revision anterior procedure was required on a patient with rheumatoid arthritis and advanced systemic disease that sustained a sacral fracture and required revision ALIF at L5/S1. CONCLUSIONS The technique described is a safe, new, muscle-splitting, psoas-preserving, one-incision approach to provide access from L1 to S1 for multilevel anterior or oblique lumbar interbody fusion surgery.

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.

Prophylactic stabilization of vertebral body metastasis at risk of imminent fracture using balloon kyphoplasty.

Study Design. Case report. Objective. Presentation of the previously unreported technique of balloon kyphoplasty being used to prophylactically stabilize a vertebral body metastasis at risk of imminent fracture. Summary of Background Data. Many patients with spinal metastases are not suitable for a total en bloc resection. Untreated these metastases may fracture, with the risk of cord compression. Methods. We present the case of a 53-year-old gentleman with an isolated T10 renal cell metastasis. This gentleman presented with back pain, and was deemed to be at risk of imminent fracture. The metastasis was prophylactically stabilized using balloon kyphoplasty. Results. Lasting pain relief and spinal stability were achieved following treatment with balloon kyphoplasty. He remains pain free 14-months post treatment. Conclusion. This is the first reported case of balloon kyphoplasty being used to prophylactically stabilize a vertebral body containing a metastasis, achieving both mechanical stability and pain relief.

Segmental pelvic correlation (SPeC): a novel approach to understanding sagittal plane spinal alignment.

BACKGROUND CONTEXT Lumbar lordosis (LL) correlates with pelvic morphology, and it has been demonstrated that as LL increases, the inflection point and apex of lordosis move cranially. This suggests that each segment of the lumbar spine relates to pelvic morphology in a unique way. OBJECTIVES This study aimed to establish whether there is a direct relationship between pelvic morphology and lumbar segmental angulation in the sagittal plane. STUDY DESIGN A retrospective analysis of 41 patient radiographs was carried out. PATIENT SAMPLE Inclusion criteria included patients with full length standing anterioposterior and lateral radiographs of the spine from base of occiput to proximal femora, with clearly visible vertebral end plates from T12 to S1 and a thoracic kyphosis (TK) and LL within the normal range. Patients were excluded if they had a coronal spinal deformity affecting the lumbar spine, chronic back pain, spondylolisthesis, spondylolysis, congenital scoliosis, or skeletal dysplasia. OUTCOME MEASURES Spinopelvic radiographic parameters of pelvic incidence (PI), LL, TK, and segmental angulation at each level from L1 to the sacrum were the outcome measures. METHODS Forty-one lateral whole spine radiographs with normal sagittal profiles from the spinal deformity clinic were retrospectively reviewed. Pelvic incidence, LL, TK, and segmental angulation at each level from L1 to the sacrum were measured (from end plate to end plate), distinguishing the vertebral body and intervertebral disc contribution. Pearson correlation coefficients were used to analyze any relationship between pelvic parameters and segmental angulation. RESULTS A strong correlation was found between PI and LL. Pelvic incidence correlated strongly with the L1 and L2 motion segments (p=.0001, p=.03), notably at the intervertebral discs but not the L4 and L5 motion segments. The proportion of total LL contributed at L4-L5 and L5-S1 reduced as PI increased. CONCLUSIONS Pelvic incidence can predict segmental angulation. Although the majority of LL is produced at the L4 and L5 motion segments, cephalad lumbar segments sequentially become increasingly important as PI increases. This describes a continuum where the L1 and L2 motion segments crucially fine-tune total LL according to PI. This allows segmental abnormalities to be identified when compensation in adjacent segments maintain normal total LL. It also paves the way for anatomical segmental reconstruction in degenerative adult deformity based on pelvic morphology.