Holocord spontaneous pneumorrhachis in the setting of refractory emesis

Background: Pneumorrhachis (PNR) is the presence of air within the spinal canal and may be either intramedullary or extramedullary in location. The etiology is most commonly iatrogenic or traumatic in nature. Treatment is dependent on underlying cause and physical exam. Case Description: Herein, we describe the second case in the literature of spontaneous holocord PNR in a young patient without risk factors. A 22-year-old male with no past medical history presented to the hospital for 2 days of vomiting and cramping in his hands and feet secondary to severe dehydration. He recently started a new job as a manual laborer and had to leave work early 2 days prior due to overexertion working outside in heat ranging from 100 to 120 degrees Fahrenheit. CT abdomen and pelvis demonstrated spontaneous pneumomediastinum and extramedullary PNR extending upward from L3 throughout the thoracic spine to the upper limit of the scan. Subsequent CT cervical and thoracic spine showed the full length of the extradural air from C2-T12 and again at L3. Conclusion: Spontaneous PNR is an uncommon, typically self-limited condition in which air is introduced into the spinal axis. Anatomic predisposition makes the extradural, dorsal cord in the cervicothoracic region the most common location. Patients are rarely symptomatic, and treatment is supportive in nature once secondary causes with high rates of morbidity and mortality are ruled out.

Extradural Glomus Tumor of the Thoracic Spine: Case Report and Literature Review

Background: Glomus tumors are rare lesions that can arise anywhere along the spinal axis. Only thirteen cases have been reported. We report a case of a patient with a rare glomangioma of the thoracic spine. Following a review of the twelve previous cases is a discussion of a spinal glomus tumor with regard to clinical presentation, diagnosis, and treatment. Methods: A single, recent case arising from the extradural space of the thoracic spine is described, followed by a literature review of spinal glomus tumors.Results: Including our case, thirteen cases of a glomus tumor have been reported. The mean age at the time of diagnosis was 44.6 years (range: 22–73 years) and the male-to-female (M:F) ratio was 1.17:1. The lesion was located in the cervical spine in 1 patient, the thoracic spine in 7 patients, the lumbar spine in 4 patients, and the sacrum in 1 patient. All the tumors were benign; however, there was one report of local recurrence because of incomplete removal. There were no malignant transformations, metastases, or deaths reported with a mean follow-up of 20.09 ± 28.43 months (range: 2–90 months).Conclusions: Spinal glomus tumor are rare, and the preoperative diagnosis is difficult. An overwhelming majority of glomus tumors are benign and are cured by simple local excision. Patients undergoing complete resection have an excellent long-term prognosis.Trial registration: Not applicable.

Extradural Glomus Tumor of the Thoracic Spine: Case Report and Literature Review

Objective Glomus tumors are rare lesions that can arise anywhere along the spinal axis. Only thirteen cases have been reported. We report a case of a patient with a rare glomangioma of the thoracic spine. Following a review of the twelve previous cases is a discussion of a spinal glomus tumor with regard to clinical presentation, diagnosis, and treatment. Methods A single, recent case arising from the extradural space of the thoracic spine is described, followed by a literature review of spinal glomus tumors. Results Including our case, thirteen cases of a glomus tumor have been reported. The mean age at the time of diagnosis was 44.6 years (range: 22–73 years) and the male-to-female (M:F) ratio was 1.17:1. The lesion was located in the cervical spine in 1 patient, the thoracic spine in 7 patients, the lumbar spine in 4 patients, and the sacrum in 1 patient. All the tumors were benign; however, there was one report of local recurrence because of incomplete removal. There were no malignant transformations, metastases, or deaths reported with a mean follow-up of 20.09 ± 28.43 months (range: 2–90 months). Conclusions Spinal glomus tumor are rare, and the preoperative diagnosis is difficult. An overwhelming majority of glomus tumors are benign and are cured by simple local excision. Patients undergoing complete resection have an excellent long-term prognosis.

Rare cause of paraparesis in a young man: cervico-dorsal neurenteric cysts associated with Klippel-Feil syndrome

Klippel-Feil syndrome is an entity presenting with short neck, low hairline and reduced range of motion of cervical spine. Neurenteric cyst is a congenital abnormality, in which mucus-secreting epithelium of the gastrointestinal tract is seen in the spinal axis. The association of a neurenteric cyst with Klippel-Feil syndrome has been reported very rarely. We report the case of a young man, affected by Klippel-Feil syndrome, who presented with bilateral paraplegia. Imaging of the spine revealed features suggestive of cervico-dorsal neurenteric cyst. Subsequently, surgical resection of the cysts was done, which resulted in resolution of the symptoms.

Diagnosis, treatment, and survival in spinal dissemination of primary intracranial glioblastoma: systematic literature review

OBJECTIVESpinal metastases from primary intracranial glioblastoma (GBM) are infrequently reported, and the disease has yet to be well characterized. A more accurate description of its clinical presentation and patient survival may improve understanding of this pathology, guide patient care, and advocate for increased inclusion in GBM research. The authors sought to describe the clinical presentation, treatment patterns, and survival in patients with drop metastases secondary to primary intracranial GBM.METHODSA systematic review was performed using the PRISMA guidelines. PubMed/MEDLINE, Scopus, Web of Science, and Cochrane databases were queried for abstracts that included patients with primary intracranial GBM and metastases to the spinal axis. Descriptive statistics were used to evaluate characteristics of the primary brain lesion, timing of spinal metastases, clinical symptoms, anatomical location of the metastases, and survival and treatment parameters. Kaplan-Meier analysis and log-rank analysis of the survival curves were performed for selected subgroups.RESULTSOf 1225 abstracts that resulted from the search, 51 articles were selected, yielding 86 subjects. The patients’ mean age was 46.78 years and 59.74% were male. The most common symptom was lumbago or cervicalgia (90.24%), and this was followed by paraparesis (86.00%). The actuarial median survival after the detection of spinal metastases was 2.8 months and the mean survival was 2.72 months (95% CI 2.59–4.85), with a 1-year cumulative survival probability of 2.7% (95% CI 0.51%–8.33%). A diagnosis of leptomeningeal disease, present in 53.54% of the patients, was correlated, and significantly worse survival was on log-rank analysis in patients with leptomeningeal disease (p = 0.0046; median survival 2.5 months [95% CI 2–3] vs 4.0 months [95% CI 2–6]).CONCLUSIONSThis study established baseline characteristics of GBMs metastatic to the spinal axis. The prognosis is poor, though these results will provide patients and clinicians with more accurate survival estimates. The quality of studies reporting on this disease pathology is still limited. There is significant need for improved reporting methods for spinal metastases, either through enrollment of these patients in clinical trials or through increased granularity of coding for metastatic central nervous system diseases in cancer databases.

Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report

Spinal and cerebellar hemangioblastomas are common in von Hippel-Lindau disease (vHLD) and usually treated surgically. Multifocal presence and surgically not amenable locations are issues that require a combined microsurgical and radiosurgical approach to control complex cases.We would like to present the case of a 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Combined microsurgical approaches to two spinal lesions and the cerebellar lesion followed by external beam radiotherapy of the posterior fossa and the whole spinal axis stabilized tumor growth of the asymptomatic lesions, while no recurrent tumors were detected at the site of surgery. A clinical deterioration connected to early postoperative deficits stabilized to a moderate gait ataxia. The follow-up after radiotherapy covered 60 months.A combination of microsurgery and radiosurgery for the surgically not amenable lesions is an adequate treatment regimen to stabilize tumor growth and clinical symptoms of multifocal spinal hemangioblastomas in vHLD, though the therapy should be limited to symptomatic or growing lesions.

Isolated Intraspinal Neurenteric Cyst, Mimicking Arachnoid Cyst: A Case Report

Neurenteric cysts account for 0.7-1.3% of spinal axis tumors. These uncommon lesions results from the inappropriate partitioning of the embryonic notochordal plate and presumptive endoderm during the third week of human development. Heterotopic nests of epithelium reminiscent of gastrointestinal and respiratory tissue lead to eventual formation of compressive cystic lesions of the pediatric and adult spine. We report a 40-year-old male presenting with slow-progressive myelopathic manifestation and ovoid non-enhancing cysticintradural extramedullary lesion at C7 level on MRI, mimicking intraspinal arachnoid cyst, who underwent successful surgical excision. The histopathological examination prove it to be neurenteric cyst.

Intrathecal pump catheter-tip granuloma recurrence with associated myelomalacia – How safe is intrathecal analgesic infusion therapy? A case report

Background: A serious complication of intrathecal (IT) infusion therapy for pain management is catheter-tip-associated granuloma. Catheter-tip granulomas can lead to permanent severe neurological sequelae if not promptly detected. Case Description: We report a patient with a recurrence of a catheter-tip granuloma causing a high-grade paresis of the lower extremities and we review briefly the literature. Conclusion: Patients with IT pump therapy presenting new neurological findings need prompt imaging of the spinal axis to rule out a catheter-tip granuloma. In case of catheter-tip granuloma, early surgical decompression is important.