Non-traumatic complete cervical spine dislocation with severe fixed kyphosis: successful multidisciplinary approach to a challenging case.

BACKGROUND: To our knowledge, there is no previous report in the literature of non-traumatic neglected complete cervical spine dislocation characterized by anterior spondyloptosis of C4, extreme head drop, and irreducible cervicothoracic kyphosis. CASE PRESENTATION: We report the case of a 33-year-old Caucasian man with a 17-year history of severe immune polymyositis and regular physiotherapy who presented with severe non-reducible kyphosis of the cervicothoracic junction and progressive tetraparesia for several weeks after a physiotherapy session. Radiographs, computed tomography, and magnetic resonance imaging revealed a complete dislocation at the C4-C5 level, with C4 spondyloptosis, kyphotic angulation, spinal cord compression, and severe myelopathy. Due to recent worsening of neurological symptoms, an invasive treatment strategy was indicated. The patient's neurological status and spinal deformity greatly complicated the anesthetic and surgical management, which was planned after extensive multidisciplinary discussion and relied on close collaboration between the orthopedic surgeon and the anesthetist. Regarding anesthesia, difficult airway access was expected due to severe cervical angulation, limited mouth opening, and thyromental distance, with high risk of difficult ventilation and intubation. Patient management was further complicated by a theoretical risk of neurogenic shock, motor and sensory deterioration, instability due to position changes during surgery, and postoperative respiratory failure. Regarding surgery, a multistage approach was carefully planned. After a failed attempt at closed reduction, a three-stage surgical procedure was performed to reduce displacement and stabilize the spine, resulting in correct spinal realignment and fixation. Progressive complete neurological recovery was observed. CONCLUSION: This case illustrates the successful management of a critical situation based on a multidisciplinary collaboration involving radiologists, anesthesiologists, and spine surgeons.

Isolated Rosai-Dorfman disease of the spine: A systematic literature review.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine. RESULTS: There were 53 patients with isolated spinal RDD (including our case). The mean age was 35.85±16.48 years. Neurological deficit was the most frequent clinical presentation (89%). RDD lesions were mainly located in the thoracic spine (51%), then the cervical spine (32%). The lesion was reported to be extradural (57%), intradural extramedullary (26%), intramedullary (7%), and in the vertebral body (10%). Histological examination showed emperipolesis in 73%. Histocytes were positive for S-100 protein in 83%. Treatment was based on surgery 96%), radiotherapy, chemotherapy, and adjunctive steroid therapy were indicated in four, one, and eight cases. After a mean follow-up period of 14.84±13.00 months, recurrence of RDD was noted in 15%. CONCLUSION: Spinal RDD is a rare condition, requiring meticulous histological examination for accurate diagnosis. Complete surgical resection is the treatment of choice. Adjuvant chemotherapy and radiotherapy can also be indicated in patients demonstrating partial improvement following surgery.

Post-operative complications affect survival in surgically treated metastatic spinal cord compression.

PURPOSE: The prevalence of metastatic epidural spinal cord compression (MESCC) is increasing globally due to advancements in cancer diagnosis and treatment. Whilst surgery can benefit specific patients, the complication rate can reach up to 34%, with limited reporting on their impact in the literature. This study aims to analyse the influence of major complications on the survival of surgically treated MESCC patients. METHODS: Consecutive MESCC patients undergoing surgery and meeting inclusion criteria were selected. Survival duration from decompressive surgery to death was recorded. Perioperative factors influencing survival were documented and analysed. Kaplan-Meier survival analysis at one year compared these factors. Univariate and multivariate Cox proportional hazard regression analyses were performed. Additionally, univariate analysis compared complicated and uncomplicated groups. RESULTS: Seventy-five patients were analysed. Median survival for this cohort was 229 days (95% CI 174-365). Surgical complications, low patient performance, and rapid primary tumour growth were significant perioperative variables for survival in multivariate analyses (p < 0.001, p = 0.003, and p = 0.02, respectively) with a hazard ratio of 3.2, 3.6, and 2.1, respectively. Univariate analysis showed no variables associated with complication occurrence. CONCLUSION: In this cohort, major surgical complications, patient performance, and primary tumour growth rate were found to be independent factors affecting one year survival. Thus, prioritizing complication prevention and appropriate patient selection is crucial for optimizing survival in this population.

Incidence of skeletal-related events in patients with Ewing sarcoma: An observational retrospective study in Japan.

BACKGROUND: Skeletal-related events (SREs), including the pathological fracture, surgical treatment or radiation of bone lesions, malignant spinal cord compression, hypercalcemia, are important considerations when managing metastatic bone tumors; however, owing to their rarity, the incidence of SREs in patients with Ewing sarcoma remains unknown. METHODS: We retrospectively reviewed the clinical data from 146 patients with Ewing sarcoma treated at a single institution from 2005 to 2019. The median age at diagnosis was 22.7 years. Fifty patients (34.2%) had metastatic disease at diagnosis. The primary outcome was the SRE-free rate among patients with Ewing sarcoma. Moreover, we identified the risk factors for SREs using univariate or multivariate analyses. RESULTS: During the observational period (median, 2.6 years), SREs occurred in 23 patients. Radiation to the bone, malignant spinal cord compression, and hypercalcemia were documented as the initial SREs in 12 patients (52.2%), 10 patients (43.5%), and one patient (4.3%), respectively. The SRE-free rate was 94.2 ± 2.0, 87.3 ± 3.0, and 79.6 ± 3.8% at 1, 2, and 3 years after the initial visit, respectively. Multivariate analysis revealed bone metastasis at diagnosis (hazard ratio [HR] = 4.41, p = 0.007), bone marrow invasion (HR = 34.08, p < 0.001), and local progression or recurrence after definitive treatment (HR = 3.98, p = 0.012) as independent risk factors for SREs. CONCLUSIONS: SREs are non-rare events that can occur during the treatment course for Ewing sarcoma, with an especially high incidence of malignant spinal cord compression. Patients with metastatic disease at diagnosis, especially in the bone or bone marrow, or with local progression or recurrence after definitive treatment, should be carefully monitored for the occurrence of SREs. The most effective methods to monitor the occurrence of SREs and new preventative therapies for SREs should be investigated in the future.

Hirayama's disease associated with cervical deformity and spinal cord compression: a case report from Sweden.

BACKGROUND: Hirayama's disease (HD) is most common in young males, and previous studies are predominantly from Asian countries. The cause of HD is unknown but the most common theory about the pathology speculates on forward bending that causes a compression of the dura mater and the anterior horn of the spinal cord against the vertebra during an overstretch flexion that may result in myelopathy. Both anterior and posterior cervical surgical approaches have been shown to be effective in stopping the disease and improving function; however, HD is also reported to be a self-limited disease, and treatment with a cervical collar may be an alternative for these patients. CASE REPORT: We report HD in a 17-year-old male from Sweden who underwent surgical treatment with a 2 level anterior cervical discectomy and fusion (ACDF) due to neurological progression from HD after conservative treatment. CONCLUSION: HD is rare and is easily overlooked. Surgical intervention shows promising results for neurological progression, but HD is also reported to be a self-limited disease.

Implementation of Robotic Exoscope in Minimally Invasive Corpectomy at Thoracolumbar Junction for the Treatment of Traumatic Spinal Cord Compression.

The development and diffusion of minimally invasive (MI) approaches have coincided with improvements in magnification systems. The exoscope will probably open a new era in new technologies in spinal surgery. This study reports a retrospective series of 19 thoracolumbar (T11-L2) burst fractures with anterior column failure and cord compression, treated with MI corpectomy and spinal decompression assisted by a three-dimensional high-definition exoscope (Video 1). Exclusion criteria were pathologic or osteoporotic fractures, multilevel fractures, and previous surgery at the site of the fracture. Three key indicators were recorded: surgical time, blood loss, and intraoperative complications. A questionnaire was administered to assess the users' exoscope experience with ergonomics, preparation, magnification, image definition, illumination, and user-friendliness, compared with the operative microscope. To the best of our knowledge, this is the first study reporting on exoscope-assisted MI corpectomy. This procedure permitted low blood loss and less surgical time without intraoperative complications. The exoscope offers clear advantages in terms of ergonomics, definition, and user-friendliness. Moreover, it is a suitable instrument for training and education, providing an opportunity for better interaction with other members of the surgical staff.

Impact of metastatic epidural spinal cord compression (MESCC) and pathological vertebral compression fracture (pVCF) in neurological and survival prognosis.

BACKGROUND: Metastatic epidural spinal cord compression (MESCC) and pathological vertebral compression fractures (pVCF) are the most serious debilitating morbidities of spine metastases (SpMs) causing devastating neurological damages. The respective impact of these two metastasis-spreading entities on survival and on neurological damage is debated. METHODS: A French prospective cohort study collected 279 consecutive patients presenting with SpMs between January 2017 and 2021. We compared 174 patients with MESCC and 105 patients with pVCF. RESULTS: The median Overall Survival (OS) for the MESCC group was 13.4 months (SD 1.5) vs 19.2 months (SD 2.3) for pVCF patients (p = 0.085). Sixty-five patients (23.3 %) were operated on: 49/65 (75.4 %) in the MESCC group and 16/65 (15.2 %) in the pVCF group, p < 0.0001. At 6 months FU, in the MESCC group, 21/44 (45.4 %) of non-ambulatory patients at onset improved to ambulatory status (Frankel D-E) vs 10/13 (76.9 %) in the pVCF group (p = 0.007). In multivariable analysis with the Cox proportional hazard model, good ECOG-PS and SINS Score 7-12 [HR: 6.755, 95 % CI 2.40-19.00; p = 0.001] were good prognostic factors for preserved ambulatory neurological status. However, SpMs diagnosed synchronously with the primary tumor [HR: 0.397, 95 % CI 0.185-0.853; p = 0.018] and MESCC [HR: 0.058, 95 % CI 0.107-0.456; p = 0.007] were independent risk factors for impaired neurological function. CONCLUSION: Contrary to pVCF, MESCC causes neurological damage. Nevertheless, neurological recovery remains possible. MESCC and pVCF have no impact on survival. The management of MESCC remains to be clarified and optimized to reduce neurological damage.

Vertebral Angiomatosis in a Dog.

A 3 yr old spayed female Cavalier King Charles spaniel was referred for insidious ataxia and paraparesis. A thoracolumbar lesion was suspected. Computed tomography showed focal osteolysis of the vertebral body and pedicles of T5. In addition, a hyperdense, extradural material within the vertebral canal, causing spinal cord compression on the right side, was present. The lesion was confirmed with magnetic resonance imaging. A T4-T5 hemi-dorsal laminectomy was performed to decompress the spinal cord. Histopathological examination was consistent with vertebral angiomatosis. After the surgery, the dog rapidly improved; however, 5 mo later the clinical signs relapsed. Vertebral angiomatosis is a vasoproliferative disorder, rarely reported as a cause of myelopathy in cats. This condition has not previously been reported in dogs. This case report describes the clinical features, the diagnostic findings, and the follow-up of a young dog with vertebral angiomatosis.

Emergency radiation therapy in modern-day practice: Now or never, or never again ?

Radiation therapy plays a fundamental role in oncological emergencies such as superior vena cava syndrome (SVCS) and metastatic epidural spinal cord compression (MESCC). These are two examples of critical complications of metastatic cancer in terms of pain and functional impact (respiratory and/or neurological). The aim of this review is to explore the current indications, treatment options and outcomes for emergency radiotherapy regarding to these complications.Regarding SVCS, studies are mostly retrospective and unanimously demonstrated a beneficial effect of radiotherapy on symptom relief. Spinal cord compression remains an indication for urgent radiotherapy, and should be combined with surgery when possible. The innovative stereotactic body radiotherapy (SBRT) showed promising results, however this technique requires small volumes and more time preparation and therefore is often unsuitable for SVCS and MESCC emergencies.This review concluded that radiotherapy has a central role to play within a multimodal approach for SVCS and MESCC treatment. Further prospective studies are needed to confirm the effectiveness of radiation and establish the criteria for selecting patients to benefit from this treatment option.

Spinal Epidural Tuberculoma with Adjacent Vertebral Fracture: A Case Report.

CASE: An 85-year-old woman was transported to our institution due to difficulty in walking. Preoperative imaging showed spinal cord lesions indicative of spinal cord tumor at the T7-8 level, accompanied by T8 vertebral fracture. Intraoperatively, the spinal lesion was suspected to be an epidural abscess; therefore, the capsule was resected, and the abscess was drained. We added pedicle screw fixation at the T6-10 level. Postoperatively, the spinal cord lesion was definitively diagnosed as spinal epidural tuberculoma. CONCLUSION: Spinal epidural tuberculomas should be considered in the treatment of spinal cord lesions causing paralysis attributed to spinal cord compression.

Spinal cord schistosomiasis in a 6-year-old child with complete recovery after spine surgery and medical treatment: case report and discussion.

Spinal cord schistosomiasis is a rare and severe form of schistosomiasis. The prognosis is largely conditioned by early diagnosis and treatment. The authors present a case of spinal cord schistosomiasis complicated by spinal cord compression syndrome. This is the case of a 6-year-old patient who presented with febrile gastroenteritis followed by complete paralysis of both lower limbs of sudden onset following a brief stay in a village setting with notion of multiple baths at a stream. Spinal cord MRI revealed an enlarged spinal cord spanning D10 to D12 with heterogeneous contrast enhancement and a syrinx cavity above the lesion. Biological workup revealed an inflammatory syndrome. Treatment consisted of decompressive laminectomy with biopsy of the lesion and a syringo-subarachnoid shunt. Pathological analysis revealed fragments of central nervous system tissues with an infiltrate composed of lymphocytes, plasmocytes, and macrophages producing granulomatous foci lined with areas of necrosis in addition to a large contingent of polynuclear eosinophils, agglutinating around or covering in some places elongated ovoid structures, with relatively thick eosinophilic shells and presenting a terminal spur. Adjuvant treatment consisted of praziquantel and corticotherapy for 1 month. The evolution showed marked improvement in the neurological deficits. She now walks unassisted and has good sphincter control. Spinal cord schistosomiasis is rare in our context; its diagnosis is difficult. The treatment is both medical and surgical.

Application of the T1w/T2w mapping technique for spinal cord assessment in patients with degenerative cervical myelopathy.

STUDY DESIGN: Retrospective case-control study. OBJECTIVES: To investigate signal changes on T1w/T2w signal intensity ratio maps within cervical cord in patients with degenerative cervical myelopathy (DCM). SETTING: Novosibirsk Neurosurgery Center, Russia. METHODS: A total of 261 patients with DCM and 42 age- and sex-matched healthy controls were evaluated using the T1w/T2w mapping method and spinal cord automatic morphometry. The T1w/T2w signal intensity ratio, which reflects white matter integrity, and the spinal cord cross-sectional area (CSA) were calculated and compared between the patients and the controls. In patients with DCM, the correlations between these parameters and neurological scores were also evaluated. RESULTS: The regional T1w/T2w ratio values from the cervical spinal cord at the level of maximal compression in patients with DCM were significantly lower than those in healthy controls (p < 0.001), as were the regional CSA values (p < 0.001). There was a positive correlation between the regional values of the T1w/T2w ratio and the values of the CSA at the level of maximal spinal cord compression. CONCLUSIONS: T1w/T2w mapping revealed that spinal cord tissue damage exists at the level of maximal compression in patients with DCM in association with spinal cord atrophy according to automatic morphometry. These changes were correlated with each other.

Treatment of ambulatory patients with metastatic epidural spinal cord compression: a systematic review and meta-analysis.

OBJECTIVE: Approximately 10% of patients with spinal metastases develop metastatic epidural spinal cord compression (MESCC), which left undiagnosed and untreated can lead to the loss of ambulation. Timely diagnosis and efficient multidisciplinary treatment are critically important to optimize neurological outcomes. This meta-analysis aimed to determine the most efficient treatment for ambulatory patients with MESCC. METHODS: The authors conducted a systematic review and meta-analysis of the treatment of mobile patients with MESCC in terms of outcomes described as local control (LC), ambulatory function, quality of life (QOL), morbidity, and overall survival (OS). RESULTS: Overall, 54 papers (4101 patients) were included. A trend toward improved LC with stereotactic body radiotherapy (SBRT) compared with conventional external beam radiotherapy (cEBRT) was demonstrated: random effects modeling 1-year LC rate 86% (95% CI 84%-88%) versus 81% (95% CI 74%-86%) (p > 0.05), respectively, and common effects modeling 1-year LC rate 85% (95% CI 82%-87%) versus 76% (95% CI 74%-78%) (p < 0.05). Surgery followed by adjuvant radiotherapy, either cEBRT or SBRT, showed no significant benefit in either LC (OR 0.88, 95% CI 0.65-1.19) or ambulatory function (OR 1.51, 95% CI 0.83-2.74) compared with radiotherapy without surgery. There was a significant benefit of surgery compared with cEBRT regarding QOL, and furthermore SBRT alone provided long-term improvement in QOL. The type of treatment was not a significant predictor of OS, but fully ambulatory status was significantly associated with improved OS (HR 0.46-0.52, relative risk 1.79-2.3). Radiation-induced myelopathy is a rare complication of SBRT (2 patients [0.1%] in the included papers). The morbidity rate associated with surgery was relatively high, with a 10% wound complication rate and 1.6% hardware-failure rate. CONCLUSIONS: SBRT is an extremely promising treatment modality being integrated into treatment algorithms and provides durable LC. In mobile patients with MESCC, surgery does not improve LC, survival, or ambulatory function; nonetheless, there is a significant benefit of surgery in terms of QOL. In patients with MESCC without neurological deficit, the role of surgery is still debatable as studies demonstrate good LC for patients who undergo SBRT without preceding surgery. However, surgery can provide safe margins for the administration of the ablative dose of SBRT to the entire tumor volume within the constraints of spinal cord tolerance. Further randomized controlled trials are needed on the benefit of surgery before SBRT in mobile patients with MESCC. With the excellent results of separation surgery and SBRT, the role of highly invasive vertebrectomy is diminishing given the complication rate and morbidity of these procedures.

Predictor of Postoperative Ambulatory Recovery in Metastatic Spinal Cord Compression with Delayed Surgical Timing and Progressive Paraplegia.

OBJECTIVE: To analyze preoperative predictors of ambulatory recovery after surgical treatment in metastatic spinal cord compression (MSCC) patients with delayed surgical timing and progressive paraplegia. METHODS: We reviewed patients with a preoperative lower-extremity motor grade of ≤3 and surgical timing ≥48 hours after the nonambulatory status. The recovery group (group R) and nonrecovery group (group NR) were classified according to ambulation assessment during follow-up. The data on patient demographics, origin of the primary tumor, pre and postoperative chemotherapy and radiation therapy, surgical procedures, Tokuhashi score, Karnofsky score, preoperative lower-extremity motor grade, and surgical timing were collected for analyzing predictors of postoperative ambulatory recovery. RESULTS: Of the 55 patients, 24 (43.6%) were group R and 31 patients were group NR. The preoperative motor grade of the lower extremities was the only predictive factor (P < 0.05). The mean hip flexor and knee extensor motor grades in group R were 2.0 ± 1.0 and 2.4 ± 1.1 respectively, while in group NR, they were 1.2 ± 1.0 and 1.3 ± 1.0. The odds ratios for failing to regain ambulatory ability were 12.6 in the knee extensor and 4.8 in the hip flexor when the motor grades 0-2 and 3 groups were compared. The rescue ratio of the preoperative hip flexor and knee extensor motor grade 0-2 group were 34.1% and 21.2%, grades 3 group were 71.4% and 77.3%, respectively. CONCLUSIONS: The significant predictive factor for ambulatory recovery was the preoperative lower-extremity motor grade. The preoperative knee extensor motor grade was identified as a more important factor than hip flexor motor grade in predicting ambulatory recovery.

Multiple spinal extradural arachnoid cysts presenting as compressive myelopathy in a teenager: case report and literature review with special emphasis on postoperative spine deformity in the current minimally invasive era.

INTRODUCTION: Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.). MATERIAL AND METHODS: We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease. RESULTS: A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique "not reported" in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up. CONCLUSIONS: Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary.