Progressive Thoracic Myelopathy Research Articles

Progressive compressive thoracic myelopathy

Extramedullary hematopoiesis is a possible complication in patients with beta-thalassemia, due to ineffective erythropoiesis, producing remote sites of hematopoiesis in multiple and very variable anatomic locations [1Gatto I. Terrana V. Biondi L. Compression of the spinal cord due to proliferation of bone marrow in epidural space in a splenectomized person with Cooley's disease.Haematologica. 1954; 38: 61-76PubMed Google Scholar, 2Haidar R. Mhaidli H. Taher A.T. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.Eur Spine J. 2010; 19: 871-878Crossref PubMed Scopus (126) Google Scholar]. Among these sites, spinal epidural extramedullary hematopoiesis carries potential risk and may lead to acute irreversible neurological deficits [[3]Salehi S.A. Koski T. Ondra S.L. Spinal cord compression in beta-thalassemia: case report and review of the literature.Spinal Cord. 2004; 42: 117-123Crossref PubMed Scopus (47) Google Scholar]. Surgery has been used as a treatment option, however there have been reports of significant blood loss [3Salehi S.A. Koski T. Ondra S.L. Spinal cord compression in beta-thalassemia: case report and review of the literature.Spinal Cord. 2004; 42: 117-123Crossref PubMed Scopus (47) Google Scholar, 4Lau S.K. Chan C.K. Chow Y.Y. Cord compression due to extramedullary hemopoiesis in a patient with thalassemia.Spine. 1994; 19: 2467-2470Crossref PubMed Scopus (35) Google Scholar, 5Garg K. Singh P.K. Singh M. et al.Long segment spinal epidural extramedullary hematopoiesis.Surg Neurol Int. 2013; 4: 161Crossref PubMed Google Scholar]. In our case, after multidisciplinary discussion, emergency surgical decompression was chosen due to the rapidly progressive paraparesis during the preceding48 hours since admission. It was felt that this would provide speedier decompression over radiotherapy. Additionally, appropriate preoperative hematologic optimization (tranexamic acid, blood and platelet transfusions) was also instigated. After induction of general anesthesia, left T5-T9 hemilaminectomies) were performed (Fig. 2). Total intraoperative blood loss was estimated at 200cc. No supplementary hematologic treatment was needed in the postoperative period. We underline the need for surgical management in cases of progressive spinal cord compression [2Haidar R. Mhaidli H. Taher A.T. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.Eur Spine J. 2010; 19: 871-878Crossref PubMed Scopus (126) Google Scholar, 4Lau S.K. Chan C.K. Chow Y.Y. Cord compression due to extramedullary hemopoiesis in a patient with thalassemia.Spine. 1994; 19: 2467-2470Crossref PubMed Scopus (35) Google Scholar]. It can be performed safely and without excessive hemorrhagic risk. In our case we used a unilateral approach which allowed complete decompression. This approach also limits blood loss and preserves soft tissue and bony structures on the contralateral side. Adequate preoperative hematologic status is essential in these cases. Radiation therapy was used as an adjuvant treatment postoperatively to prevent recurrence of epidural extramedullary hematopoiesis. Follow-up was performed at 6 weeks and 3 months. Complete neurologic recovery was observed, with no recurrence at 3 months MRI (Fig. 3). In conclusion, the optimal management for extramedullary hematopoiesis with neurological impairment remains unproven and is currently based on case-by-case situations. We suggest that surgical management can be safely performed with optimal hematologic preparation without incurring excessive intraoperative blood loss. Note 1: A signed patient consent form has been supplied for publication. Note 2: Posthumous authorship for Dr Christian Wider.

P.119 Minimally invasive surgical disconnection of a spinal dural arteriovenous fistula with the use of intraoperative digital subtraction angiography

Background: Spinal dural arteriovenous fistulas (dAVF) are a significant but treatable cause of progressive myelopathy. The goal of treatment is disconnection of the fistula, which is often accomplished through an open surgical approach. We report two cases using a minimally invasive surgical (MIS) approach for dAVF ligation with intraoperative digital subtraction angiography (DSA) to confirm occlusion. Methods: Case report. Results: Two patients presented with progressive thoracic myelopathy and were identified to have fistulous connections at the left L1 and T8 levels respectively. Intraoperatively, a left femoral puncture was performed and a 5-French (40 cm) sheath was inserted. Patients were positioned prone and intraoperative spinal DSA was performed using the Siemens Zeego. Once the feeding radicular artery was visualized, image overlay and cross-hair laser was used to trace and localize the fistulous zone. A unilateral single level MIS hemi-laminectomy was performed. The fistulous zone and accompanying nerve root were exposed and small hemostatic clips were applied followed by surgically disconnection. Finally, intraoperatively video angiography as well as spinal DSA were performed for confirmation. Conclusions: MIS disconnection with intraoperative DSA is a safe and effective technique for treating spinal dAVFs. Patients benefit from quicker recovery and shorter hospital stay.

Idiopathic Spinal Cord Herniation—Case Report

AbstractIdiopathic spinal cord herniation is a rare cause of progressive myelopathy, especially in the absence of a history of spinal or surgical trauma. The radiological diagnosis is made through a myelography or an MRI exam. The spinal cord is pushed anteriorly, buffering the dural defect and leading in most cases to Brown-Séquard syndrome. The present study describes the case of a male patient with a clinical picture of progressive thoracic myelopathy. In the clinical and radiological investigation, an idiopathic spinal cord herniation on the chest level was identified. During the surgery, the spinal cord was reduced to the natural site, taking its usual elliptical shape, and the dural defect was repaired with a dural substitute. The numbness of the patient improved, and the shocks in the lower limbs disappeared. A postoperative MRI confirmed the surgical reduction of the herniation and the restoration of the anterior cerebrospinal fluid (CSF) column to the spinal cord. The authors describe the clinical, radiological, intraoperative, and postoperative evolution.

Percutaneous Decompression and Fixation on Complex Spinal Metastases

Materials and Methods Retrospectively analyzed the postoperative results of a series of 6 patients with vertebral metastases, with complicated mechanical pain and neurological involvement using a decompression technique and percutaneous fixation. Discuss the results with the literature. Results Total of 6 cases, 3 men and 3 women. Average age: 67.8 years (66–85), all with known primary tumor: 2 lungs tumor, 2 breast tumor, 1 prostate tumor and 1 renal tumor. Neurological compromise: L5 radicular pain paresis and one progressive thoracic myelopathy. SINS: 8,9,10,12,13,13. Tomita: 3,5,5,6,10,10. Surgical technique: posterolateral decompression, tube 5 Kapener decompression tube type 1, all percutaneous pedicle fixation. Complications: 1 patient L5 lumbar epidural hematoma resolved with surgical evacuation. Five of six went to early radiotherapy Evolution: In all of them neurological recovery and relief of mechanical and radicular pain. Conclusions Conventional treatment of patients with neurologic involvement secondary to vertebral metastasis is surgical decompression and open setting, this depending on the vital prognosis and patient status as mayor surgeries. The high rate of complications and fixing decompression percutaneous seem to offer an effective alternative in the management of the complication with minimal complications.

Thoracic Vertebral Actinomycosis Secondary to a Pulmonary Origin

Actinomycosis is a chronic infection caused by a gram-positive anaerobic bacteria from the species Actinomycesand causes a chronic colliquative inflammatory reaction known as actinomycotic granuloma, which is characterized macroscopically by suppuration, sinus tract formation, and purulent discharge containing yellowish sulfur granules. It can invade any part of the human body. This is a case report of a 40-year-old male patient known to the cardiothoracic team due to a sarcoma of the left lung. He presented with progressive thoracic myelopathy. Initially, the diagnosis was thought to be a spinal metastasis from the lung lesion. Further investigation revealed a thoracic actinomycosis with epidural granuloma tissue causing a spinal compression.

Microendoscopic posterior decompression for the treatment of thoracic myelopathy caused by ossification of the ligamentum flavum: a technical report.

Ossification of the ligamentum flavum (OLF) is a common cause of progressive thoracic myelopathy in East Asia. Good surgical results are expected for patients who already show myelopathy. Surgical decompression using a posterior approach is commonly used to treat OLF. This study investigated the use of microendoscopic posterior decompression for the treatment of thoracic OLF. Microendoscopic posterior decompression was performed on 9 patients with myelopathy. Patients had a mean age of 59.8years and single-level involvement, mostly at the T10-11 and T11-12 vertebrae. Computed tomography and magnetic resonance imaging were used to classify the OLF. A tubular retractor and endoscopic system were used for microendoscopic posterior decompression. Midline and unilateral paramedian approaches were performed in 2 and 7 patients, respectively. Intraoperative motor evoked potentials (MEPs) of 7 patients were monitored. Pre- and postoperative neurological status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score. Thoracic OLF for all patients were classed as bilateral type with a round morphology. Improvement of MEPs at least one muscle area was recorded in all patients following posterior decompression. A dural tear in one patient was the only observed complication. The mean recovery rate was 44.9%, as calculated from mJOA scores at a mean follow-up period of 20months. Microendoscopic posterior decompression combined with MEP monitoring can be used to treat patients with thoracic OLF. The optimal surgical indication is OLF at a single vertebral level and of a unilateral or bilateral nature, without comma and tram track signs, and a round morphology.

Pediatric iatrogenic thoracic kyphosis and tension myelopathy treated with a thoracic pedicle subtraction osteotomy: a case report and review of the literature

Pediatric kyphotic deformity is an uncommon clinical entity that can occur following posterior spinal operations and has significant complexity in its treatment. Tension myelopathy in a pediatric patient with a thoracic kyphotic deformity has not been reported in the literature. We present a 17-year-old boy with a progressive thoracic kyphosis and tension myelopathy 4years after he underwent a thoracic laminoplasty at an outside institution for the treatment of a dorsal spinal arachnoid cyst. At our institution, he was treated with a pedicle subtraction osteotomy as well as thoracic Ponte osteotomies for sagittal plane correction to relieve the tension myelopathy. Both clinical and radiographic improvements were observed after surgery. We review the literature on pediatric thoracic kyphosis and tension myelopathy and the treatment of these pathologies.

Idiopathic spinal cord herniation: Clinical review and report of three cases

Idiopathic spinal cord herniation (ISCH) is a rare condition, of unknown pathogenesis, that primarily affects the thoracic spinal cord. It is characterized by ventral displacement of the spinal cord through a dural defect. The aim of this study was to review the literature and to present a retrospective single center experience with three cases of ISCH. The literature review analyzed a total of 78 relevant publications on ISCH, which included a total of 171 patients, supplemented with 3 patients treated at our institution. Numerous case reports have demonstrated improvement in clinical outcomes after surgery; however, follow-up is predominantly short, data are incomplete, the condition is frequently misdiagnosed, and it is difficult to predict which patients will benefit from surgery. We identified 159 cases treated with surgical management. The mean symptom duration was 54 months, and the mean follow-up 33 months. The result at follow-up was improved neurological outcome in 74%, unchanged result in 18%, and worse outcome in 8%. There were 15 cases of conservative management, with mean symptom duration 52 months and mean follow-up 33 months. The neurological outcome was unchanged in 100%. ISCH is a rare condition causing progressive thoracic myelopathy, and the natural history is unknown. There is a lack of evidence-based treatment strategies, and the majority of cases are treated with surgical management at diagnosis. Management of ISCH needs to be individualized for each patient, and clinicians should be encouraged to report new cases, standardize case reports, and ensure long-term follow-up.

MRI Features of Spinal Solitary Fibrous Tumors

Spinal solitary fibrous tumors (SFT) are very rare neoplasms occurring in the spinal canal, with only 38 cases reported in ten years since the first description. We describe two cases of SFT of the spine and review 33 well-documented cases in the literature to define distinctive radiological and surgical features raising the suspicion of a spinal SFT before histological verification. A 67-year-old man with cervical myeloradiculopathy had a large extramedullary tumor of the cervical spinal canal extending from C4 to C7. On MRI the tumor was isointense on T1-sequences and hypointense on T2-sequences, and had marked contrast enhancement. At surgery, the tumor was intradural extramedullary, with no dural or root attachment, but it was adherent to the cord. Complete tumor removal was achieved with good outcome. A 75-year-old man with progressive thoracic myelopathy had an intramedullary tumor at C6 and C7 level, which was hypointense on T1- and T2-weighted images of MRI. At surgery, the tumor was intramedullary and strongly adherent to the cord; it was successfully removed. Both tumors were composed of elongated cells with a collagen-matrix background. Immunohistochemical staining was positive for vimentin, CD34, and bcl-2, and negative for EMA and S-100 protein. A careful analysis of our own and the other reported cases of spinal SFTs may disclose some peculiar features of this rare tumor. A spinal intramedullary or extramedullary tumor, hypointense on T2-weighted images of MRI, which intraoperatively shows hard consistency, scarce vascularization, no nerve root involvement, no or weak dural attachment, absence of arachnoidal interface, and adherence to the spinal cord may suggest the diagnosis of SFT.

Progressive thoracic myelopathy caused by spinal calcium pyrophosphate crystal deposition because of proximal junctional vertebral compression fracture after lumbopelvic fusion

We describe cases presenting with progressive thoracic myelopathy after lumbopelvic fusion attributed to proximal junctional vertebral compression fracture (PJF) followed by spinal calcium pyrophosphate dehydrate (CPPD) crystal deposition. The study included six patients, ranging from 62 to 75 years. All patients had been treated previously with lumbopelvic fusion. The mean period from the detection of PJF to the onset of myelopathy was 4.8 months. Notably, five patients demonstrated upper-instrumented vertebra (UIV) collapse. After revision surgery involving decompressive laminectomy and extension of the spinal fusion, all patients experienced significant improvement. Photomicrographs of the resected ligamentum flavum showed CPPD crystals and multinucleated giant cells. The combination of mechanical stress plus PJF and CPPD crystal deposition followed by a foreign body reaction to the deposited crystals caused myelopathy. Patients with radiographic evidence of PJF, especially UIV collapse, after lumbopelvic fusion should be followed carefully for the emergence of myelopathy.

Spinal cord herniation: management and outcome

Spinal cord herniation: management and outcome

The value of intraoperative motor evoked potential monitoring during surgical intervention for thoracic idiopathic spinal cord herniation

Thoracic idiopathic spinal cord herniation (TISCH) is a rare neurological disorder characterized by an incarceration of the spinal cord at the site of a ventral dural defect. The disorder is associated with clinical signs of progressive thoracic myelopathy. Surgery can withhold the natural clinical course, but surgical repair of the dural defect bears a significant risk of additional postoperative motor deficits, including permanent paraplegia. Intraoperative online information about the functional integrity of the spinal cord and warning signs about acute functional impairment of motor pathways could contribute to a lower risk of permanent postoperative motor deficit. Motor evoked potential (MEP) monitoring can instantly and reliably detect dysfunction of motor pathways in the spinal cord. The authors have applied MEPs during intraoperative neurophysiological monitoring (IOM) for surgical repair of TISCH and have correlated the results of IOM with its influence on the surgical procedure and with the functional postoperative outcome. The authors retrospectively reviewed the intraoperative neurophysiological data and clinical records of 4 patients who underwent surgical treatment for TISCH in 3 institutions where IOM, including somatosensory evoked potentials and MEPs, is routinely used for spinal cord surgery. In all 4 patients the spinal cord was reduced from a posterior approach and the dural defect was repaired using a dural graft. Motor evoked potential monitoring was feasible in all patients. Significant intraoperative changes of MEPs were observed in 2 patients. The changes were detected within seconds after manipulation of the spinal cord. Monitoring of MEPs led to immediate revision of the placement of the dural graft in one case and to temporary cessation of the release of the incarcerated spinal cord in the other. Changes occurred selectively in MEPs and were reversible. In both patients, transient changes in intraoperative MEPs correlated with a reversible postoperative motor deficit. Patients without significant changes in somatosensory evoked potentials and MEPs demonstrated no additional neurological deficit postoperatively and showed improvement of motor function during follow-up. Surgical repair of the dural defect is effected by release and reduction of the spinal cord and insertion of dural substitute over the dural defect. Careful monitoring of the functional integrity of spinal cord long tracts during surgical manipulation of the cord can detect surgically induced impairment. The authors' documentation of acute loss of MEPs that correlated with reversible postoperative motor deficit substantiates the necessity of IOM including continuous monitoring of MEPs for the surgical treatment of TISCH.

Diffuse arachnoid ossification and multiple arachnoid cysts presenting with progressive thoracic myelopathy

An ossified arachnoid membrane combined with cystic formation is rarely reported as a cause of spinal cord compression. We report the case of a 60-year-old man who presented with diffuse ossification of the arachnoid membrane (arachnoid ossification) and multiple cystic changes (arachnoid cyst) at the thoracic and lumbar spine. The lesions were surgically removed and progressive deterioration was prevented, although no marked improvement of neurological symptoms was attained.

Thoracic ventral dural defect: Idiopathic spinal cord herniation

Thoracic ventral dural defect, and resultant idiopathic spinal cord herniation, is a rare but increasingly recognized cause of a chronic progressive thoracic myelopathy, particularly in middle-aged women. A neurosurgically confirmed case is presented, together with a review of the pathogenesis, clinical presentation, imaging features, treatment options and progress of this entity post-treatment.

Spinal cord astrocytoma presenting as “idiopathic” intracranial hypertension

Increased intracranial pressure is rarely seen in association with spinal tumors. We describe a young, non-obese man who presented with increased intracranial pressure, papilledema and visual obscuration. Multiple cerebrospinal fluid (CSF) examinations with normal or minimally elevated CSF protein lead to the initial diagnosis of idiopathic intracranial hypertension. After a lumboperitoneal shunt placement a progressive thoracic myelopathy developed 7 months after onset of symptoms. The spinal MRI showed a low cervical-upper thoracic intramedullary tumor. Open biopsy confirmed a grade 3 fibrillary astrocytoma. The suspected mechanisms of spinal tumors causing increased intracranial pressure are reviewed as well as three other cases of spinal astrocytomas previously reported in the literature that presented with papilledema and increased intracranial pressure without hydrocephalus. This case illustrates that increased intracranial pressure may in exceptional cases of spinal tumors precede the more typical myelopathic presentation by months and mimic idiopathic intracranial hypertension.

Solitary Thoracic Intramedullary Schwannoma Report of Two Cases

Schwannomas are the most common tumors of the spinal cord. Nevertheless, sporadic intramedullary location of this entity is a very rare condition. We present two cases of solitary thoracic intramedullary schwannoma, located at the T6 and T10 levels respectively, in patients without evidence of Von Recklinghausen's disease. Two males, 71 and 51 years old respectively, presented with signs and symptoms of progressive thoracic myelopathy. In both cases, magnetic resonance imaging disclosed a spinal cord tumor, located at T6 and T10 levels respectively. Both patients were treated surgically. Patient 1 underwent an operation via a posterolateral extrapleural approach. The second patient was submitted to surgical resection of the tumor via a posterior approach. In both patients, total excision of an intramedullary lesion was achieved and histological and immunohistochemical findings were consistent with a typical schwannoma. Postoperative magnetic resonance imaging demonstrated no evidence of residual tumor in either case. The first patient is currently symptom-free and neurologically intact, almost five years after surgery. About one year after operation, patient 2 presents nearly complete resolution of his motor and sensory disturbances. Solitary intramedullary schwannomas are rare. Controversy remains regarding the origin of these tumors. In the majority of the cases, intramedullary schwannomas are benign, non-infiltrating tumors, hence amenable to aggressive surgery. Radical excision remains the gold standard in the treatment of such lesions.

Idiopathic anterior spinal cord hernia: under-recognized cause of thoracic myelopathy

Until recently, idiopathic anterior spinal cord herniation was thought to be vanishingly rare. It is an increasingly recognized, readily treatable, condition causing progressive thoracic myelopathy. We have operated on five cases and know of two other patients locally who are as yet undecided on surgery. We are aware of several other unpublished cases in the UK. The syndrome presents typically in middle age with a history of stepwise slowly progressive mid-thoracic anterior hemicord dysfunction characterized by hemianalgesia below the affected segment followed by asymmetric lower limb hypertonia, with initial sparing of posterior column sensation, that progresses through paraparesis to complete paraplegia. The radiological findings, although subtle, are equally typical and surgical repair is easily effected. With increasing clinical and radiological awareness, we believe the apparent incidence of this condition will increase. Idiopathic anterior spinal cord hernia is more common than currently appreciated, and should be positively considered and excluded in every case of progressive thoracic cord dysfunction.

Extensive arachnoid ossification with associated syringomyelia presenting as thoracic myelopathy

The authors present the case of a patient in whom progressive thoracic myelopathy was caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and the results of a literature search, they describe a pathological basis of this rare condition, discuss its incidence and symptomatology, and suggest a simple classification of various types of the arachnoid ossification. They also discuss magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. Emphasis is placed on the particular value of plain computerized tomography, which is highly sensitive for detecting intraspinal calcifications and ossifications, in the diagnostic evaluation of patients whose clinical picture indicates progressive myelopathy.

Paraplegia following post-traumatic thoraci spinal stenosis: A case report

Compromise of the spinal canal and its neural clements is a well-recognized pathological entity affecting the lumbar or cervical spine. Thoracic stenosis in the absence of a generalized rheumatological, orthopedic, or metabolic disorder is rare. The authors report a case of progressive thoracic myelopathy leading to paraplegia following severe thoracic spinal stenosis secondary to post-traumatic hypertrophy of thoracic laminae and ossification of the ligamentum flavum and posterior longitudinal ligament.

The variable presentations of craniocervical and cervical dural arteriovenous malformations

The authors reviewed four patients with dural arteriovenous malformations in the upper spinal axis. Two were at the foramen magnum and two were lower cervical. The patients presented with subarachnoid hemorrhage, a slowly progressive cervical myelopathy, a rapidly progressive thoracic myelopathy, and tinnitus with a sixth nerve palsy. This report emphasizes the importance of studying both the intracranial dural vessels as well as the supply to the cervical spine in searching for a spinal arteriovenous malformation. Subarachnoid hemorrhage with negative cerebral angiography requires spinal angiography if there are any signs or symptoms suggesting cord or nerve root dysfunction. Embolization by an endovascular approach resulted in an angiographic cure in two patients. A combination of embolization and surgery resulted in obliteration of the arteriovenous malformation in one patient. Embolization achieved a clinical cure in one patient, and clinical improvement in two patients.