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.

Surgical Outcomes of Extensive Dome-Like Laminoplasty Using En Bloc Resection of C2 Inner Lamina for Patients With Severe Cord Compression Behind C2 Body.

STUDY DESIGN: A retrospective, single-center study. OBJECTIVE: The aim of this study is to evaluate the efficacy and safety of a newly developed extensive dome-like laminoplasty using en bloc resection of the C2 inner lamina in patients with severe cord compression behind the C2 body. SUMMARY OF BACKGROUND DATA: A surgery for severe cord compression behind C2 body is challenging for spinal surgeons. To date, there has been no established solution for severe cord compression behind the C2 body. MATERIALS AND METHODS: Patients with severe cord compression behind the C2 body who underwent posterior surgery consecutively were enrolled. Extensive dome-like laminoplasty that was newly developed was performed to remove en bloc removal of the C2 inner lamina were performed. Preoperative and postoperative canal diameters behind the C2 and mean removed area of the C2 inner lamina were measured using MRI and CT scan. Clinical and radiographic parameters were assessed preoperative and postoperative periods. In addition, perioperative complications were analyzed. RESULTS: A total of 36 patients underwent extensive dome-like laminoplasty and their diagnoses were ossification of the posterior longitudinal ligament (OPLL, 66.7%) and congenital stenosis with spondylosis (33.3%). The mean canal diameter behind the C2 increased from 9.85 (2.28) mm preoperatively to 19.91 (3.93) mm at the last follow-up ( P <0.001). Clinically, neck and arm visual analog scale, Japanese Orthopaedic Association score, and neck disability index significantly improved at postoperative 1 month ( P <0.05), and the scores were maintained until the last follow-up. No meaningful radiographic changes occurred after the surgeries. During the procedures, there were no particular complications, but one patient showed deteriorated myelopathic symptoms and underwent additional C1-C2 decompressive surgery. CONCLUSIONS: After extensive dome-like laminoplasty, surgical outcomes are satisfactory, and complications are rare. This technique may be a viable option for patients with severe cord compression behind the C2 body. LEVEL OF EVIDENCE: Level IV.

Capillary Hemangioma of the Spinal Cord: Case Report and Systematic Review of Literature.

BACKGROUND: Capillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with intramedullary extension rare. We present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of the literature. METHODS: Medical records and imaging data were retrospectively reviewed using the health record software EPIC (Verona, Wisconsin, USA) and the radiology management software system RIS/PACS (Radiology Information System/Picture Archiving and Communication System; QREADS). The report was written in accordance with the CARE (case reports) guidelines. We also performed a systematic review of the literature on all cases of intramedullary spinal capillary hemangiomas in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. RESULTS: We report a case of a 54-year-old man who presented with progressive paraplegia and sensory deficits in the lower extremities. Spinal magnetic resonance imaging showed an intramedullary enhancing lesion centered at T11 with associated spinal cord compression. He underwent thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological examination. Histological examination showed findings consistent with a capillary hemangioma. The literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangioma. CONCLUSIONS: Purely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature. These should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients.

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.

Residual volume of extruded disc material and residual spinal cord compression measured on postoperative MRI do not predict neurological outcomes in dogs following decompressive surgery for thoracolumbar intervertebral disc extrusion.

Published studies on the validity of using quantitative MRI measures of pre- and postoperative spinal cord (SC) compression as prognostic indicators for dogs undergoing surgery for intervertebral disc extrusion (IVDE) are currently limited. The aim of this retrospective analytical study was to describe the volume of postoperative residual extradural material (VREM) and the ratio of the cross-sectional area (CSA) of maximum SC compression to the CSA of SC in a compression-free intervertebral space as MRI measures of preoperative and postoperative compression (residual spinal cord compression, RSCC), and to compare these measures between the neurological outcome in a group of dogs. Inclusion criteria were dogs that underwent surgery for thoracolumbar IVDE, were imaged pre- and immediately postoperatively by MRI, and had a neurological follow-up examination 2 to 5 weeks postoperatively. Two blinded observers independently performed measurements in pre- and postoperative MRI studies. Dogs were classified into positive outcome (PO) and negative outcome (NO) groups based on follow-up neurologic examination scores. Seventeen dogs were included (12 PO, 5 NO). Interobserver agreement for MRI measurements was good to excellent (ICCs: 0.76-0.97). The prevalence of residual extradural material in postoperative MRI studies was 100%. No significant differences in mean preoperative SC compression, mean RSCC, mean SC decompression, or VREM were found between outcome groups (P = .25; P = .28; P = .91, P = .98). In conclusion, neither postoperative VREM nor RSCC could predict successful neurological outcomes.

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.

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.

Two pediatric observations of spinal extradural arteriovenous fistulas presenting with epidural hemorrhages and cord compression.

We report two cases of acute spinal cord compression in children with low-flow spinal epidural arteriovenous fistulas (SEAVFs) and discuss the clinical presentation and management of these vascular anomalies. While most low-flow SEAVFs without radiculomedullary drainage are benign lesions typically diagnosed incidentally, we suggest that asymptomatic lesions may warrant aggressive management in specific circumstances, including lesions diagnosed at an early age or in patients under anticoagulation therapy. Our observations also emphasize that patients with a "spontaneous" epidural hemorrhage should undergo dedicated preoperative or postoperative vascular imaging to identify a possible underlying vascular anomaly.

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.

Extensive, circumferential, epidural spinal cord compression by IgG4-related pachymeningitis.

A rare case of IgG4-related pachymeningitis is presented. The manuscript describes the multidisciplinary treatment of a rare entity in an exceptional location, prompting emergent surgical treatment. However, the correct intraoperative hypothesis led to a minimal surgical approach with fewer risks and good disease control. Finding a balance between maximising treatment outcome and minimising risks is often difficult for clinicians worldwide who encounter the same clinical dilemma. The known cases are summarised, as are radiological characteristics and common treatments.

Investigation of perfusion impairment in degenerative cervical myelopathy beyond the site of cord compression.

The aim of this study was to determine tissue-specific blood perfusion impairment of the cervical cord above the compression site in patients with degenerative cervical myelopathy (DCM) using intravoxel incoherent motion (IVIM) imaging. A quantitative MRI protocol, including structural and IVIM imaging, was conducted in healthy controls and patients. In patients, T2-weighted scans were acquired to quantify intramedullary signal changes, the maximal canal compromise, and the maximal cord compression. T2*-weighted MRI and IVIM were applied in all participants in the cervical cord (covering C1-C3 levels) to determine white matter (WM) and grey matter (GM) cross-sectional areas (as a marker of atrophy), and tissue-specific perfusion indices, respectively. IVIM imaging resulted in microvascular volume fraction ([Formula: see text]), blood velocity ([Formula: see text]), and blood flow ([Formula: see text]) indices. DCM patients additionally underwent a standard neurological clinical assessment. Regression analysis assessed associations between perfusion parameters, clinical outcome measures, and remote spinal cord atrophy. Twenty-nine DCM patients and 30 healthy controls were enrolled in the study. At the level of stenosis, 11 patients showed focal radiological evidence of cervical myelopathy. Above the stenosis level, cord atrophy was observed in the WM (- 9.3%; p = 0.005) and GM (- 6.3%; p = 0.008) in patients compared to healthy controls. Blood velocity (BV) and blood flow (BF) indices were decreased in the ventral horns of the GM (BV: - 20.1%, p = 0.0009; BF: - 28.2%, p = 0.0008), in the ventral funiculi (BV: - 18.2%, p = 0.01; BF: - 21.5%, p = 0.04) and lateral funiculi (BV: - 8.5%, p = 0.03; BF: - 16.5%, p = 0.03) of the WM, across C1-C3 levels. A decrease in microvascular volume fraction was associated with GM atrophy (R = 0.46, p = 0.02). This study demonstrates tissue-specific cervical perfusion impairment rostral to the compression site in DCM patients. IVIM indices are sensitive to remote perfusion changes in the cervical cord in DCM and may serve as neuroimaging biomarkers of hemodynamic impairment in future studies. The association between perfusion impairment and cervical cord atrophy indicates that changes in hemodynamics caused by compression may contribute to the neurodegenerative processes in DCM.

Kinematic alteration in three-dimensional reaching movement in C3-4 level cervical myelopathy.

OBJECT: This study aimed to compare the reaching movement between two different spinal cord compression level groups in cervical myelopathy (CM) patients. METHODS: Nine CM patients with maximal cord compression at the C3-4 level (C3-4 group) and 15 CM patients with maximal cord compression at the C4-7 level (C4-7 group) participated in the study. We monitored three-dimensional (3D) reaching movement using an electronic-mechanical whack-a-mole-type task pre-and post-operatively. Movement time (MT) and 3D movement distance (MD) during the task were recorded. An analysis of variance for split-plot factorial design was performed to investigate the effects of compression level or surgery on MT and MD. Moreover, we investigated the relationship between these kinematic reaching parameters and conventional clinical tests. RESULTS: The 3D reaching trajectories of the C3-4 group was unstable with higher variability. The C3-4 group showed longer MT (p < 0.05) and MD (p < 0.01) compared with the C4-7 group both before and after surgery. Moreover, MT was negatively correlated with the Japanese Orthopedic Association score only in the C3-4 group (r = - 0.48). CONCLUSION: We found that spinal cord compression at the C3-4 level had a negative effect on 3D reaching movement and the kinematic alteration influenced the upper extremity performance. This new knowledge may increase our understanding of kinematic alteration in patients with CM.

Magnetic resonance imaging characteristics of atlanto-axial subluxation in 42 dogs: Analysis of joint cavity size, subluxation distance, and craniocervical junction anomalies.

Background: Atlanto-axial (AA) subluxation can be a complex syndrome in dogs. Accurate identification and assessment of this condition are key to providing treatment and resolution. Aim: The purpose of this retrospective study was to describe the magnetic resonance imaging (MRI) characteristics of AA subluxation and associated neurologic deficits. Methods: A multicenter review of dogs with a diagnosis of AA subluxation was conducted, evaluating signalment, neurologic grade, duration of signs, and MRI characteristics. MRI characteristics included degree of spinal cord compression and joint subluxation, integrity of odontoid ligaments, presence of a dens, spinal cord signal intensity, and presence of syringohydromyelia, hydrocephalus, and Chiari-like malformation. A control population with normal AA joints was also evaluated. MR images of 42 dogs with AA subluxation were compared to 26 age and breed-matched control dogs. Results: Affected dogs had a median age of 27 months and a median weight of 2.7 kg, and the most commonly affected breed was the Yorkshire terrier (47.5%). Spinal cord signal hyperintensity, increased AA joint size, and cross-sectional cord compression at the level of the dens and mid-body C2 were associated with AA subluxation. No associations were found between cord compression, the appearance of the dens, or cord signal intensity and neurologic grade. Affected dogs did not have a higher incidence of Chiari-like malformation, syringohydromyelia, or hydrocephalus than control dogs, and their neurologic grade was not associated with MRI findings. Lack of dens and/or odontoid ligaments was associated with larger subluxations. Conclusion: Dogs with clinical signs of AA subluxation were significantly more likely to have intramedullary hyperintensity at the level of compression ( p = 0.0004), an increased AA joint cavity size ( p = 0.0005), and increased spinal cord compression at the level of dens and mid-body C2 (p ≤ 0.05). The authors suggest an AA joint cavity size >1.4 mm and a subluxation distance >2.5 mm as cutoffs for MRI diagnosis of AA subluxation in dogs. No differences were noted between dogs with AA subluxation and control dogs regarding syringohydromyelia, hydrocephalus, and Chiari-like malformation.

Metastatic paraganglioma presenting with spinal cord compression requiring urgent surgery.

We report a rare case of a functional bladder paraganglioma diagnosed in a young man who presented with acute compressive thoracic myelopathy secondary to vertebral metastasis. A histological diagnosis of a metastatic paraganglioma was made following biopsy of a rib lesion. CT revealed a lesion in the inferior wall of the bladder, which demonstrated avid uptake on 68Ga-DOTATATE PET/CT. Serum metanephrine levels were more than 40 times the upper limit of normal. The patient was hypertensive and treatment with doxazosin was initiated. In view of neurological deterioration, he required urgent spinal decompression to preserve neurological function and prevent permanent paraplegia. Despite inadequate alpha-blockade, surgery was successful, and the perioperative course was uneventful. Alpha-blockade was subsequently optimised. Treatment with cyclophosphamide, vincristine and dacarbazine was started but, in view of disease progression, treatment was subsequently changed to sunitinib.

Spinal metastases and metastatic spinal cord compression

This guideline covers recognition, referral, investigation and management of spinal metastases and metastatic spinal cord compression (MSCC). It is also relevant for direct malignant infiltration of the spine and associated cord compression. It aims to improve early diagnosis and treatment to prevent neurological injury and improve prognosis.

An unusual cause of chronic neuropathic pain: report of a case of multiple intradural spinal arachnoid cysts and review of the literature.

Spinal arachnoid cysts (SACs) arise either intra- or extradurally and are usually solitary, while cases of multiple SACs have been scarcely reported in the literature. Herein, we report on a rare case of multiple and recurring intradural spinal arachnoid cysts (SACs) causing severe spinal cord compression and neuropathic radicular pain, in a 35-year-old female with a 10-year follow-up. Two separate attempts at surgery were performed but only provided temporary relief since the cysts recurred and new ones formed along the entirety of the spinal cord. Finally, a conservative approach with physiotherapy and a combination of analgesic medications was pursued.

Dynamic cord compression induced by proximal junctional failure and loose pedicle screws after thoracolumbar fusion surgery: a case report.

BACKGROUND: One of the common mechanical complications following spinal fusion surgery is proximal junctional failure (PJF). The incidence of neurological deficit associated with PJF has been poorly described in the literature. Here, we report a case in which numbness in the lower extremities was recognized as the first symptom, but the discrepancy in the imaging findings made PJF difficult to diagnose. METHODS: A 71-year-old female underwent corrective fusion surgery. Three weeks later, she complained of persistent right leg numbness. Standing X-ray showed the back-out of the pedicle screws (PSs) in the upper instrumented vertebra (UIV), but there was no obvious evidence of cord compression on computed tomography (CT), which caused the delay of diagnosis. Five weeks later, magnetic resonance image (MRI) did not show cord compression on an axial view, but there were signal changes in the spinal cord. RESULTS: The first reason for the delayed diagnosis was the lack of awareness that leg numbness could occur as the first symptom of PJF. The second problem was the lack of evidence for spinal cord compression in various imaging tests. Loosened PSs were dislocated on standing, but were back to their original position on supine position. In our case, these contradictory images led to a delay in diagnosis. CONCLUSION: Loosened PSs caused dynamic cord compression due to repeated deviation and reduction. Supine and standing radiographs may be an important tool in the diagnosis of PJF induced by dynamic cord compression.