Turbulent Cerebrospinal Fluid Flow in Enlarging Terminal Myelocystocele.

Terminal myelocystocele is a rare type of neural tube malformation, consisting of a skin-covered lumbosacral mass, highly associated with other complex abdominal malformations within the OIES complex (omphalocele, imperforate anus, exstrophy of the cloaca and spinal defects). We present a case of a premature female with an extensive lumbosacral mass at birth, as well as an omphalocele, cloacal exstrophy, renal abnormalities, and sacral agenesis. Lumbar magnetic resonance imaging revealed a meningocele sac herniating through the bone defects and containing a syringocele sac. Advanced imaging techniques showed turbulent cerebrospinal fluid flow. At control, 4 weeks later, the defect doubled in size. The myelocystocele sac was evacuated and closed, and the patient persisted with paraparesis. The role of cerebrospinal fluid flow analysis is well established in Chiari-type malformations, in which turbulent flow within the syrinx is related to a better outcome after surgery. It is possible that the same principle could be applied to other spinal malformations, as shown in this case of terminal myelocystocele.

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

Primary Lumbar Paraganglioma: Clinical, Radiologic, Surgical, and Histopathologic Characteristics from a Case Series of 13 Patients.

BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors, rarely occurring in the lumbar spine. Primary lumbar paragangliomas are prominently vascularized, can present variably, and pose both diagnostic and surgical challenges. We report on a large case series with long-term follow-up and intraoperative footage to characterize the natural history, diagnostic approach, and operative approach to this rare surgical disease. METHODS: This is a single-center, retrospective cohort study including all patients with histologically confirmed primary lumbar paraganglioma treated at our tertiary neurosurgical center between 1997 and 2018. Clinical, radiologic, surgical, and histologic data were collected from medical records. RESULTS: There were 13 cases of primary lumbar paraganglioma (8 men [61.5%], 5 women [38.5%]; mean age, 51.3 years; range, 33.2-68.9 years). Symptom duration correlated with tumor size (Spearman r = 0.735, P = 0.01). The main presenting symptoms were lower back pain and radiculopathy, often long-standing with recent deterioration. Seven patients (53.8%) were admitted as emergency cases, including 3 with cauda equina syndrome. Preoperative differential diagnoses included nerve sheath tumor, ependymoma, meningioma, and disk herniation. The mean Ki-67 mitotic index was 5.7% (range, 1%-10%). Surgical resection improved pain in 8 of 13 patients (61.5%) and weakness improved in 5 of 5 patients (100%). CONCLUSIONS: Primary lumbar paragangliomas are rare neoplasms of the cauda equina that typically progress slowly but may also present acutely. They are often related to the filum terminale, which should be resected prior to other attachments intraoperatively to prevent displacement of the tumor out of view. Total resection can be curative, and long-term follow-up in this series found no recurrence.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin (POEMS) changes syndrome presenting with a pseudosensory level: a case report.

INTRODUCTION: Polyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm. We report the first case of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome presenting with a pseudosensory level. CASE PRESENTATION: A 59-year-old Tamil woman with long-standing diabetes mellitus and hypertension developed painless, progressive inguinal lymphadenopathy. A contrast-enhanced computed tomography scan showed mild hepatomegaly and intra-abdominal lymphadenopathy. A histological examination of an enlarged inguinal lymph node showed features of a plasma cell-type Castleman disease. She was treated with rituximab. Six months later, she developed gradually ascending numbness and weakness of both lower limbs. On examination, she had flaccid paraparesis (power 3/5) with a sensory level to pinprick at thoracic level 9. Joint position sense was preserved. Her cranial nerves and upper limbs were neurologically normal. Nerve conduction studies confirmed peripheral neuropathy with conduction slowing and a magnetic resonance imaging of her spine did not show cord or root compression. Serum protein electrophoresis showed a monoclonal band. A bone marrow biopsy showed a hypercellular marrow with 30% plasma cells. A repeat contrast-enhanced computed tomography scan showed sclerotic bony lesions involving multiple vertebrae in addition to mild hepatomegaly and intra-abdominal lymphadenopathy. Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome was diagnosed and she was treated with intravenously administered pulse therapy of dexamethasone and cyclophosphamide. After three cycles of treatment, she regained normal muscle power and sensation. CONCLUSIONS: Polyneuropathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome can present as a pseudosensory level.

Transradial approach in the treatment of a sacral dural arteriovenous fistula: a technical note.

Sacral dural arteriovenous fistulas (SDAVFs) are rare, constituting no more than 10% of all spinal dural fistulas. They are most commonly fed by the lateral sacral artery (LSA), a branch of the internal iliac artery (IIA). Catheterization of this vessel requires either a crossover at the aortic bifurcation in cases of right femoral access or retrograde catheterization from the ipsilateral common femoral artery. We present the case of a 79-year-old man with tethered cord syndrome and a symptomatic SDAVF fed by two feeders from the left LSA. Spinal diagnostic angiography was made exceptionally challenging by an aorto-bi-iliac endograft, and selective catheterization of the left IIA was not possible. The patient could not undergo surgery due to multiple comorbidities, therefore embolization was considered the best approach. The procedure was carried out through a transradial access (TRA) with Onyx and n-butyl cyanoacrylate. The SDAVF was successfully treated and the patient made a full neurological recovery.

Acute Paraparesis Due to Protrusion of a Disc Following Lateral Interbody Fusion for Degenerative Kyphoscoliosis: A Case Report.

CASE: A 74-year-old woman presented with severe trunk deformity. Radiographs revealed severe sagittal and coronal imbalance with spinal canal stenosis at L4 to L5. Anterior cages were placed at L2 to L3, L3 to L4, and L4 to L5. Three days later, posterior correction surgery from T5 to the ilium with decompression at L4 to L5 was performed. At 30 minutes after surgery, leg muscle strength severely deteriorated. Emergency surgery revealed disc fragments protruding into the spinal canal at L2 to L3. CONCLUSION: Because posteriorly placed extreme lateral interbody fusion (XLIF) cages can be a risk factor for disc protrusion into the spinal canal, computed tomographic evaluation or prophylactic posterior decompression should be considered before the correction procedure.

Intervertebral disc herniation in two coatis (Nasua nasua) and postoperative laminectomy membrane formation.

Magnetic resonance imaging revealed spinal cord compression due to intervertebral disc herniation of Hansen type I and II in the thoracolumbar vertebral column in two middle-aged coatis (Nasua nasua) with chronic progressive paraparesis. Surgical treatment included hemilaminectomy and partial corpectomy in one and dorsal laminectomy in the other coati. Both coatis recovered well after surgery. One showed unremarkable gait 6 and 15 months post surgery, while the other one suffered from recurrence of paraparesis leading to euthanasia because of deterioration of neurological signs 20 months after the first surgery. Necropsy revealed formation of a laminectomy membrane compressing the spinal cord. Histopathological signs of spinal cord injury and findings of degenerative processes in the intervertebral disc were comparable to those described in dogs. In conclusion, this case report shows for the first time that surgical intervention seems to be a useful and safe treatment in chronic intervertebral disc herniation in coatis, but relapses are possible.

The effect of intercostal artery reimplantation on spinal cord injury in thoracoabdominal aortic aneurysm surgery.

OBJECTIVE: Intercostal artery (ICA) reimplantation (ICAR) is thought to decrease spinal cord injury (SCI) in thoracic aortic aneurysm and thoracoabdominal aortic aneurysm (TAAA) surgery. Patients treated from 1989 to 2005 without ICAR were compared with those treated from 2005 to 2013 with ICAR to determine whether ICAR reduced SCI. We hypothesized that ICAR would reduce SCI, especially in the highest-risk patients. METHODS: This was a retrospective analysis using a prospectively maintained Investigational Review Board-approved database from a university tertiary referral center. The analysis included all patients (n = 805) undergoing thoracic aortic aneurysm and TAAA surgery from 1989 to 2013. The main outcome measure was any transient or permanent paraplegia or paraparesis (SCI). From 1989 to 2004, ICAR was not performed in patients, and open ICAs were ligated; from 2005 to 2013, open ICAs at T7 to L2 were reimplanted in patients with Crawford type I, II, and III TAAAs. Surgical technique was cross clamp without assisted circulation. Anesthetic management was the same from 1989 to 2013. Demographic, intraoperative, and outcome variables were assessed by univariate and multivariate analysis. Observed/expected ratios for paralysis were calculated. RESULTS: A total of 540 patients had surgery before 2005, and 265 had surgery after 2005, when ICAR was begun. There were 275 type I, II, and III TAAAs before 2005 and 164 after 2005. Aneurysm extent, acuity, SCI, mortality, renal failure, and pulmonary failure were the same in patients treated before and after 2005. Multivariate modeling of all patients showed type II TAAA (P = .0001), dissection (P = .00015), and age as a continuous variable (P = .0085) were significant for SCI. Comparing only type I, II, and III TAAAs, there was no difference in SCI between those with ICAR after 2005 and those without ICAR before 2005 (5.1% vs 8.8%; P = .152). In a subanalysis of the highest-risk patients (type II, dissection, acute), ICAR was not significant (P = .27). Observed/expected ratios ratios were 0.23 before 2005 and 0.16 after 2005 (χ2 = .796; P = .37). CONCLUSIONS: Although there was a small decrease in SCI with ICAR, reattaching ICAs did not produce a statistically significant reduction in SCI, even in the highest-risk patients.

IgG4-related disease presenting with an epidural inflammatory pseudotumor: a case report.

BACKGROUND: Inflammatory pseudotumor is a rare clinical condition that can be related to immunoglobulin G4 disease. Only a few cases of spinal inflammatory pseudotumors have been reported in the literature and an association with immunoglobulin G4 disease was not conclusive in any of them. We describe what we believe to be the first biopsy-proven case of an epidural inflammatory pseudotumor related to immunoglobulin G4 disease. CASE PRESENTATION: A 57-year-old Caucasian woman presented to our hospital with severe paraparesis, gait disturbance, and sensory loss secondary to a relapsing epidural mass. Examination of a biopsy specimen revealed a lymphoplasmacytic infiltration with fibrosis and an immunoglobulin G4-positive plasma cell ratio of over 50 %, which are compatible with a diagnosis of immunoglobulin G4-related inflammatory pseudotumor. Our patient was successfully treated with systemic and epidural administration of glucocorticoids. CONCLUSION: Immunoglobulin G4-related disease is an emerging clinical condition in which central nervous system involvement is still uncommon. We describe the case of a patient with an epidural mass with medullar compression, which was proved to be an immunoglobulin G4-related epidural inflammatory pseudotumor. Our findings suggest a new manifestation of immunoglobulin G4-related disease. This disorder should be considered in the differential diagnosis of spinal tumors as a potentially treatable condition with glucocorticoids.

Development of intraspinal ectopic endometrial tissue in connection with tethered cord syndrome.

We herein report a rare case of intraspinal ectopic endometrial tissue associated with tethered cord syndrome. The patient underwent MR imaging of the lumbar spine and CT spinal angiography. Asymptomatic dysraphism was also detected, including spinal bifida, low-lying conus medullaris, spinal meningocele and a lumbosacral lipoma. Venous reflux obstruction was also suggested. The patient underwent L2-S1 laminectomy and microdecompression of the lesion. The histological and immunohistochemical features were characteristic of ectopic endometrial tissue. Since the surgery, no neurological signs have been noted, either during or outside the patient's menstrual periods. The two-month follow-up MRI scans showed a regression of the lesion.

Complete recovery from paraparesis in spinal cord compression due to extramedullary haemopoiesis in beta-thalassaemia by emergency radiation therapy.

Extramedullary haemopoiesis (EMH) is a complication commonly associated with beta-thalassaemia intermedia; it is frequently asymptomatic but can sometimes lead to symptomatic tumour-like masses. No guidelines or common consensus are available in literature regarding the different treatment strategies and only single cases have been reported. We describe a case of spinal cord compression due to intrathoracic EMH masses treated with combined radiotherapy and hydroxyurea.

Thoracic disc herniation leads to anterior spinal artery syndrome demonstrated by diffusion-weighted magnetic resonance imaging (DWI): a case report and literature review.

BACKGROUND: Thoracic disc herniation rarely causes acute ischemic events involving the spinal cord. Few reports have suggested this as a mechanism leading to anterior spinal artery syndrome, and none with illustration through diffusion-weighted magnetic resonance imaging (DWI). PURPOSE: The purpose of this study was to report a case of anterior spinal artery syndrome secondary to thoracic disc herniation and demonstrate the first use of DWI to aid in diagnosis of this rare myelopathy. STUDY DESIGN: Case report. METHODS: A 36-year-old woman developed sudden onset of back pain followed by evolving paraparesis and sensory loss consistent with anterior spinal artery distribution ischemia. T2-weighted magnetic resonance imaging (MRI) demonstrated an acute herniated nucleus pulposus at the T7-T8 disc, which produced a focal indentation of the adjacent anterior spinal cord without cord displacement or canal stenosis. T2-weighted hyperintensities were seen at T4-T7 levels with corresponding brightness on DWI and reduction of the apparent diffusion coefficient, consistent with cord ischemia. RESULTS: Remarkably, within just a few days and following conservative treatment, including heparin and steroids, this patient's neurologic status began to show improvement. Within 3 weeks, she was ambulating with assisted devices, and at the 10-month follow-up, the patient had nearly complete neurological improvement. A follow-up MRI at 10 months showed normal T2-weighted imaging except for a 1×2-mm area of anterior-left lateral cord myelomalacia at T4-T5. CONCLUSIONS: Acute thoracic disc herniation with cord contact but without canal stenosis is able to disrupt blood flow to the cord leading to anterior spinal artery distribution ischemia. This case represents the first demonstrated use of DWI in diagnosing this rare cause of anterior spinal artery ischemia.

Trehalose protects against spinal cord ischemia in rabbits.

OBJECTIVE: This study tested to see if trehalose, a cytoprotective disaccharide, protects against spinal cord ischemia in a rabbit model. METHODS: The infrarenal aorta was mobilized in four groups of 10 rabbits. In groups I, II, and III, it was clamped proximally and distally for 20 minutes. In group I, the clamped aorta was infused at 2.5 L/min for 2 hours with lactated Ringer's (LR) solution. In group II, the clamped aorta was infused with 5% trehalose in LR. LR was administered intravenously (2.0 mL/min) in groups I and II starting 30 minutes before clamping. In group III, 5% trehalose in LR was infused intravenously only. Group IV was a sham-operated control group without aortic clamping. At 8, 24, and 48 hours after reperfusion, hind limb function was scored using the Tarlov score (paralysis = 0, perceptible joint movement = 1, good joint movement but unable to stand = 2, able to walk = 3, normal = 4). Histologic analysis and electron microscopy were performed on anterior horn cells. RESULTS: The Tarlov scores in groups I, II, and III were, respectively, 1.1 ± 1.4, 3.5 ± 0.5, and 2.9 ± 0.9 at 8 hours; 0.8 ± 1.2, 3.9 ± 0.3, and 2.9 ± 0.9 at 24 hours; and 0.6 ± 0.7, 3.9 ± 0.3, and 2.7 ± 0.9 at 48 hours after reperfusion. Group IV scores were normal (4 ± 0) at all assessments. These scores were higher in groups II and III than in group I (P < .01) at all assessments. Scores at 24 and 48 hours were higher in group II than in group III (P < .05). In group III, delayed paraparesis developed in one rabbit at 24 hours and in two more at 48 hours. Histopathologic analysis showed the number of normal neurons was higher in groups II (P < .0001), III (P = .006), and IV (P < .0001) vs group I. Electron microscopy confirmed preserved neuronal cell ultrastructure in rabbits with normal limb function. CONCLUSIONS: Transaortic trehalose infusion was protective against paraplegia, whereas intravenous trehalose reduced spinal cord ischemia. This study was preliminary and further studies are needed.

Spinal cord function monitoring during endovascular treatment of thoracoabdominal aneurysms: implications for staged procedures.

AIM: Spinal cord ischemia is a well-known complication in the treatment of thoracoabdominal aneurysms (TAAA). Despite the fact that endovascular treatment of TAAA is less invasive, spinal cord ischemia rate is not reduced if compared to open repair. METHODS: We report the results of our experience of spinal cord function monitoring by measuring motor evoked potentials (MEP) during endovascular treatment of TAAA type II and III. Depending on the level of the MEPs the decision is made whether to stage the procedure or not. We treated ten patients according to this protocol. RESULTS: In two patients, MEPs decreased 50% or more and procedures were staged. Both experienced no neurological complications after first and second procedure. No MEPs decrease was seen during the second procedures. One of the other eight patients had a temporary right lower leg pararesis. CONCLUSION: In conclusion we state that our first experience demonstrates the value of assessing spinal cord function during extensive endovascular TAAA repair with subsequent strategies to prevent paraplegia.

Surgical and endovascular treatment of pediatric spinal arteriovenous malformations.

OBJECTIVE: Pediatric spinal arteriovenous malformations (AVMs) are rare and complex lesions to treat. There are few reports of the endovascular and microsurgical treatment of these lesions in the pediatric population, and the treatment outcomes of these patients are not well described. The aim of this study was the clinical and radiographic outcomes of spinal AVMs in pediatric patients treated via endovascular and microsurgical modalities. METHODS: We identified nine children (5 boys, 4 girls; average age 11 years, range 3-17 years) treated for spinal AVMs between 1998 and 2010. Their charts were reviewed. RESULTS: Spinal AVMs most frequently involved the thoracic spinal cord. Four patients had associated Klippel-Trènaunay-Weber syndrome and one had hereditary hemorrhagic telangiectasia. There were two intramedullary, four conus medullaris, and three mixed extradural-intradural lesions. The most common presenting signs and symptoms were subarachnoid hemorrhage (n = 3) and paraparesis (n = 5). Endovascular intervention was used exclusively in two patients, and combined endovascular and microsurgical intervention was used in four patients. Surgery was the sole treatment in three patients with excellent results. There were two treatment-related complications: one case of subarachnoid hemorrhage and one case of scrotal swelling. The mean follow-up was 28.5 months and the median was 8 months (range, 1-65 months). The mean pretreatment World Health Organization (WHO)/Zubrod score was 2.4 (range, 1-4), and the mean post-treatment score was 1.4 (range, 0-4). One patient (11%) had a recurrence. CONCLUSIONS: Pediatric spinal AVMs require complex combined microsurgical and endovascular techniques to achieve favorable outcomes.

[Importance of sagittal alignment in spinal revision surgery]. / Bedeutung des sagittalen Profils bei Revisionseingriffen an der Wirbelsäule.

Ignoring the sagittal profile in primary spinal fusion surgery is a common reason for revision surgery. Therefore, it is important that in cases of spinal revision surgery the sagittal alignment is realized. The physiological alignment of the instrumented spine should also indirectly influence the profile of the non-stabilized spine cranial and caudal to the fusion. Patients with normal C7 plumb-line and a physiological sacral inclination have a lower incidence of adjacent segment degeneration. Sagittal imbalance after revision surgery is a risk factor for recurrent pseudarthrosis. In cases of pseudarthrosis a combined approach may be more effective in realizing sagittal balance und enhancing rates of fusion.

Painless transient paraparesis as the solitary manifestation of aortic dissection.

We present a 62-year-old man with acute and temporary paraparesis of the lower extremities as the solitary symptom of an anterior spinal artery syndrome caused by a type B aortic dissection. Ischemia of the spinal cord was confirmed by magnetic resonance imaging. Neurologic symptoms resolved completely within 6 hours and conservative treatment was successful up to 8 months follow-up. Our report illustrates that painless, transient neurologic deficit can be the only presenting symptom of acute aortic dissection and that aortic dissection should be part of the differential diagnosis of acute paraparesis.

Reversal of delayed-onset paraparesis after revision thoracic endovascular aortic repair for ruptured thoracic aortic aneurysm.

Thoracic endovascular aortic repair (TEVAR) is an important surgical option for the emergency treatment of ruptured thoracic aortic aneurysms, but is associated with a risk of spinal cord ischemia (SCI). Although risk factors for the development of SCI have been well described, the effectiveness of treatment to increase spinal cord perfusion pressure remains incompletely understood. We report the successful treatment of delayed-onset paraparesis after revision TEVAR for acute descending thoracic aortic rupture with the combined use of blood pressure augmentation and cerebrospinal fluid drainage. The clinical manifestations, pathophysiology, and management of SCI after TEVAR are reviewed.