Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: Case report and review of the literature

Context

Hemangioblastomas of the spinal cord are uncommon vascular tumors. Patients commonly present with subtle neurologic findings that are thought to represent growth of the lesion over time. Hemorrhage of an intramedullary hemangioblastoma presenting as acute neurologic deficit is an extremely rare occurrence. Although the cervical spine is the most common location for hemangioblastoma of the spinal cord, there have been no previously published cases in the literature of intramedullary hemorrhage from such a lesion.

Findings

A 22-year-old woman with a previously undiagnosed spinal cord hemangioblastoma presented with sudden-onset dense quadriparesis due to intramedullary hemorrhage in the cervical spinal cord. The patient did not have any clinical findings of von-Hippel Lindau disease. Laminoplasty from C5 to T2 and posterior midline myelotomy for resection of the intramedullary tumor with hematoma evacuation were completed without complication.

Conclusion

Intramedullary hemangioblastoma of the spinal cord is uncommon, and hemorrhage from a cervical spinal cord lesion has not previously been reported. Symptoms from these usually indolent lesions are commonly associated with tumor growth, edema, or associated syrinx, whereas devastating acute neurologic deficit from hemorrhage is exceedingly rare. Microsurgical resection should be done in cases of symptomatic lesions and considered in isolated symptomatic lesions without the known diagnosis of von Hippel-Lindau disease.     

Sarcoidosis presenting as an isolated intramedullary tumor

We report a case of isolated intramedullary sarcoidosis. The patient developed progressive signs that indicated a spinal tumor, which were investigated with contrast-enhanced magnetic resonance imaging scans. Magnetic resonance imaging clearly revealed an intramedullary lesion, but the diagnosis of sarcoidosis was made on the pathological analysis of the surgical specimen. Magnetic resonance imaging with contrast enhancement is reported in a histologically proven case of intramedullary sarcoidosis. Only 12 other cases of isolated intramedullary sarcoidosis have been reported. We review and discuss these cases according to their clinical presentation, the segmental location of the granulomas in the spinal cord, preoperative and operative diagnoses, and signs for systemic sarcoidosis. In none of the cases was the diagnosis of intramedullary sarcoidosis made before surgery. We think that surgical therapy for intramedullary lesions is the best way to diagnose rare instances of benign lesions like sarcoidosis and to treat them in an appropriate manner.     

A mobile hemangioblastoma of the cauda equina: Case report and review of the literature

Context: Cases of migratory spinal tumors have been reported since 1963. Most involve spinal schwannomas, which are benign tumors of the lining of nerve cells. We report a rare case of a mobile spinal hemangioblastoma, which is a type of benign vascular tumor.

Findings: A 50-year-old man visited the hospital for painful swelling in his lower back. An MRI scan indicated that a lesion was at the L5 vertebral level. Two weeks later, however, an enhanced MRI showed that the lesion had migrated to the L4 vertebral level. During surgery, the location of the lesion remained consistent with the enhanced MRI reviewed. The histopathological diagnosis was hemangioblastoma.

Conclusion: This is the first known report of a mobile spinal hemangioblastoma. Mobile spinal hemangioblastoma requires careful preoperative and intraoperative evaluation of its real-time location to avoid performing surgery at the wrong vertebral level.     

Dermoid cyst: A rare intramedullary inclusion cyst

Intramedullary dermoid cysts are rare tumors, especially those not associated with spinal dysraphism. Only six cases have been reported in the literature. Of these, only two cases have had magnetic resonance imaging studies. We report a case of an 18-year-old female patient, who presented with progressive weakness of both the lower limbs and wasting of both the upper limbs. Magnetic resonance imaging (MRI) showed an intramedullary lesion extending from C3 to D2 with peripheral enhancement on contrast. Decompression of the cystic contents with partial removal of cyst wall was done. Hair with oily cholesterol and keratin debris was encountered. Histopathology confirmed the diagnosis of dermoid cyst. This case adds to the previous reported cases of the rare and uncommon intramedullary space occupying lesions of the spinal cord.     

Intramedullary spinal cord metastasis associated with hemorrhage: a case report

A rare case of intramedullary spinal cord metastasis associated with hemorrhage was reported. A 74-year-old man had a subacute onset of paraparesis. He became almost paraplegic within a few days. MRI revealed an intramedullary spinal lesion in the epiconus at the Th 11 and Th 12 level, but spinal angiography did not show any abnormality. Since repeated MRI showed enlargement of the lesion, surgery was performed under the diagnosis of an intramedullary spinal cord tumor. Under general anesthesia, a midline myelotomy of about 3 cm was performed and a grayish, elastic and circumscribed tumor as well as a liquefied hematoma in the caudal part was observed. Both the tumor and the hematoma were removed almost totally. The patient's paraparesis improved slightly after surgery. The histological diagnosis was adenocarcinoma. The primary source was unknown, but multiple small metastatic tumors were found in the lung, liver and brain, etc. Hemorrhage from intramedullary spinal cord metastasis is extremely rare with only 6 reported cases in the recent literature. Rapid deterioration of symptoms caused by the hematoma may make the diagnosis more difficult. Indication of surgical treatment should be carefully determined because prognosis of intramedullary spinal cord metastasis is generally very poor.     

Intractable hiccups caused by syringobulbia and syringomyelia associated with intramedullary spinal hemangioblastoma

Introduction

Hiccups caused by a neoplasm in the spinal cord are rare.

Materials and methods

We report a case of intractable hiccups caused by syringobulbia and syringomyelia associated with cervical intramedullary spinal hemangioblastoma, which was successfully treated by surgical excision. A 60-year-old man was referred from the neurology department after presenting with hiccups for 1 year. The hiccups were aggravated 3 months ago and were sustained during eating or sleeping. Several doctors administered a muscle relaxant and an anticonvulsant, but they were ineffective. Spinal MRI revealed a huge syringomyelia from C2 to T2, associated with a highly enhancing intramedullary mass lesion at the C5 level. The hiccups were ceased after removal of the tumor through a right hemilaminectomy. The pathology of the specimen was hemangioblastoma. The size of the syringobulbia and syringomyelia decreased markedly on MRI checked 5 months after surgery.

Conclusions

Intractable hiccups can be caused by syringobulbia associated with an intramedullary cord tumor in the cervical area and possible mechanisms of hiccups were reviewed.

     

Bilateral Mid-Diaphyseal Femoral Stress Fractures in the Elderly

Abstract Background: Osteoporosis is characterized by low bone density and poor bone mineralization. Mid-diaphyseal stress (insufficiency) fractures of the femur caused by osteoporosis are rare. The symptoms of these lesions are vague and confusing. The physician must be alert to the possibility of femoral shaft stress fractures when evaluating an elderly patient complaining of back, hip or leg pain. Case Study: A 72-year-old osteoporotic female patient with bilateral mid-diaphyseal stress fractures of the femur is reported. The diagnosis of femoral shaft stress fracture in the elderly is very difficult when based on physical findings and plain radiography only. A magnetic resonance imaging (MRI) scan or nuclear scintigraphy is necessary for early diagnosis. In the described case, the patient had an excellent result after surgical treatment with intramedullary nails. Conclusion: MRI or nuclear scintigraphy must be obtained to exclude the possibility of femoral shaft stress fractures in the elderly. In the absence of contraindications, surgery is the best solution for this kind of lesion.     

Remote medulla ablongata ventral acute subarachnoid hemorrhage following cervical spinal surgery: A case report

IntroductionThe incidence of remote intracranial hemorrhage (RICH) in patients during spinal surgery is rare and the detailed mechanism remains unclear.Presentation of caseA 55-year-old man had undergone cervical discectomy and fusion at C5–6 and C6–7 due to herniated disc and secondary spinal canal stenosis. He had severe headache 20 h postoperatively and his drain output increased from 100 to 350 mL in the second 10 h after surgery. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed and he was diagnosed with acute subarachnoid hemorrhage in the ventral medulla oblongata. The drainage tube was quickly removed. Infusion of hypertonic saline was used to reduce intracranial pressure and nimodipine prevented vasospasm around the brainstem. The patient made a gradual, satisfactory recovery with conservative treatment.DiscussionThe most likely pathomechanism leading to RICH is venous bleeding due to rapid leak of a large amount of cerebral spinal fluid (CSF) after spinal surgery. If the patient has a headache or neurological complaints after spinal surgery, immediate imaging is recommended to confirm the diagnosis. Treatment depends on the amount and location of intracranial hemorrhage.ConclusionRICH is a serious but rare complication of spinal surgery and cerebellar hemorrhage is the most common. The most important pathomechanism leading to RICH after spinal surgery is venous bleeding due to rapid leak of a large amount of CSF. Timely CT is necessary to exclude RICH. Treatment of RICH depends on the size of the intracranial hematoma and the patient’s symptoms.     

Visualization of vascular structure of spinal hemangioblastoma using intraoperative indocyanine green videoangiography and temporary feeder occlusion

Background and purpose

To more safely resect pathological lesions during spinal vascular lesion surgery, it is most important to understand local abnormal hemodynamics in detail. New devices or techniques that make out intraoperative local hemodynamics have been awaited. To introduce a resourceful method, we present a case of spinal hemangioblastoma for which temporary arterial occlusion during near-infrared intraoperative indocyanine green (ICG) videoangiography gives useful assessment of the main and minor feeders easily.

Methods

A 36-year-old female suffered progressive paresthesia of both lower extremities for 12 months and gait disturbance for 2 weeks. A neurological examination revealed T10 myelopathy. Magnetic resonance imaging (MRI) of the thoracic spine showed an intramedullary tumor at the T8 level and severe spinal cord edema with a flow void in the extended dorsal spinal veins. Spinal angiography showed a hemangioblastoma at the T8 level, with two main feeders and minor feeders.

Results

She underwent total resection of the tumor by a posterior approach. During the intraoperative ICG videoangiography, temporary arterial occlusion of the two main feeders and FLOW^®800 analysis enabled clear understanding of the vasculature, especially of the two minor feeders. At the 9-month follow-up, her neurological manifestation was partially resolved, and post-operative MRI showed total removal of the tumor and disappearance of the spinal cord edema.

Conclusions

Temporary clipping of the main feeders during intraoperative ICG videoangiography is very useful for easily determining the minor feeding arteries, and helpful for maintaining normal perfusion of the spinal cord in spinal hemangioblastoma surgery. Furthermore, the FLOW 800 analysis, especially the false color-coded variation, increased our understanding of the hemodynamics.

     

Intramedullary spinal cord metastasis from renal cell carcinoma mimicking intramedullary hemangioblastoma

A rare case of intramedullary spinal cord metastasis from renal cell carcinoma mimicking intramedullary hemangioblastoma was described. A 57-year-old man had a nephrectomy for renal cell carcinoma in the left kidney 5 years ago, and no recurrence or metastasis was found in computed tomography and bone scintigraphy. Later, the patient was presented as having bilateral shoulder pain and severe palsy of bilateral upper and lower extremities, and a solitary tumor in the intramedullary spinal cord was found at the C4 level. Excision of the tumor was performed and the intraoperative pathological diagnosis suspected the tumor to be hemangioblastoma. However, a final pathological examination revealed characteristics consistent with metastasis of renal cell carcinoma. Although the patient’s neurological condition and neuralgia initially slightly deteriorated postoperatively, they then gradually improved. Twenty-two months after the operation, a follow-up magnetic resonance imaging showed no recurrence of intramedullary spinal cord tumor, and there were no other metastases found in other organs.     

Lumbar spinal stenosis in a young individual as a result of ligamantum flavum ossification: A case report

INTRODUCTIONSymtomatic lumbar ligamantum flavum calcification is quite rare in the young age group.PRESENTATION OF CASEThe authors report a case of young adult with diagnosis of lumbar spinal stenosis, presenting with leg pain and neurological deficits. Computerized tomography (CT) scan and magnetic resonance (MR) imaging studies revealed ossification of the ligamantum flavum as the causative factor of the disease and the patient recovered completely after the decompressive operation.DISCUSSIONIt is emphasized that attention should be given to this rare etiological factor of lumbar spinal stenosis.CONCLUSIONComplete relief can be achieved with early and adequate surgery.     

Interesting case of subependymoma of the spinal cord

Background contextSubependymomas are rare, slow-growing, and usually noninvasive/nonaggressive World Health Organization Grade I tumors that tend to occur in the ventricles. Their most common site of occurrence is the fourth ventricle followed by the lateral ventricles. Spinal cord subependymomas typically manifest as cervical and cervicothoracic intramedullary or, rarely, extramedullary mass lesions. They often present clinically with pain and neurologic symptoms, including motor, sensory, urinary, and sexual dysfunction. Histologically, there are hypocellular areas with occasional clusters of cells and frequent microcystic changes, calcifications, and hemorrhage. Radiologically, subependymomas generally manifest as eccentric well circumscribed nodular lesions with mild-to-moderate enhancement.PurposeTo highlight an interesting and rare presentation for subependymoma of the spinal cord.Study designThis is a case report of a single patient in whom a subependymoma was resected from the cervical spinal cord with return to normal functioning.MethodsClinical examination, magetic resonance imaging evaluation, surgical resection, and histological analysis were performed for diagnosis and treatment of this patient.ResultsThe patient experiencing myelopathy symptoms underwent a surgical resection of cervical spinal cord subependymoma that resulted in return to normal function.ConclusionsSubependymoma should be included in the differential diagnosis of atypical presentations for myelopathy, as discrete surgical resection can result in good outcome.     

Hodgkin's disease of the thoracic vertebrae

Background contextHodgkin's disease rarely occurs in the spine, which is usually a setting for the advanced form of the disease.PurposeTo describe an unusual case of isolated, primary spinal Hodgkin's disease and to draw attention to this disease as a possible diagnosis in patients with mixed inflammatory cell infiltrate lesions located in the thoracic spine.Study design/settingA case report of a 28-year-old woman who presented with back pain and progressive weakness in the lower extremities as a result of spinal cord compression from Hodgkin's disease of the thoracic vertebrae.MethodsWe report a new case of spinal cord compression resulting from Hodgkin's disease of the thoracic vertebrae. Decompression surgery was performed in the patient, followed by antibiotic treatment.ResultsAntibiotic therapy temporarily improved inflammation and fever. However, magnetic resonance imaging (MRI) evaluation showed that the inflammatory reaction in the lesion was not completely resolved. The disease progressed and later investigations revealed Hodgkin's disease, which improved with a course of chemotherapy and radiation.ConclusionsHodgkin's disease should be considered in the differential diagnosis of spinal neoplastic lesions with clinical features similar to spondylitis. Because MRI evaluation showed that the vertebral disc was maintained in this case, the presence of a tumor rather than inflammation should have been suspected.     

Re-aggravation of myelopathy due to intramedullary lesion with spinal cord enlargement after posterior decompression for cervical spondylotic myelopathy: serial magnetic resonance evaluation

STUDY DESIGN: A case report. OBJECTIVES: To report a case of swelling of the spinal cord and an intramedullary lesion occurring after expansive laminoplasty for cervical spondylotic myelopathy. SETTING: A university hospital in Japan. METHODS: Clinical evaluation, radiography, MR imaging. RESULTS: A 65-year-old man with a cervical spondylotic myelopathy in whom symptoms were improved immediately after expansive laminoplasty, but became aggravated 2 weeks later. Magnetic resonance images demonstrated swelling of the spinal cord and an intramedullary lesion that extended from the medulla oblongata to C7. Nine months after surgery, the lesion was reduced to C2-6, but neurological deterioration had not improved. Six years after surgery, the patient remains confined to bed. CONCLUSION: Patients with such disease conditions are rare, and it is difficult to predict postoperative swelling of the spinal cord before surgery. Spine surgeons should be aware of such rare disease conditions involving the spinal cord.     

Arteriovenous malformation at the cervicomedullary junction: A case report with magnetic resonance imaging

A rare case of arteriovenous malformation at the cervicomedullary junction is reported. A 72-year-old man developed symptoms of total transection of the upper cervical cord without evidence of subarachnoid hemorrhage. The arteriovenous malformation had an intramedullary nidus with an aneurysmal dilatation at the C-1 level. This lesion was diagnosed by magnetic resonance imaging and confirmed at autopsy. The neurological symptoms may have been due to compression of the spinal cord by the intramedullary lesion, followed by degeneration and necrosis. The usefulness of magnetic resonance imaging in the neuroradiological diagnosis is particularly emphasized.     

Spinal intramedullary arachnoid cyst: case report and literature review

Background contextIntramedullary arachnoid cysts are extremely rare; only 14 cases have been reported in the literature so far.PurposeWe report on the case of a 31-year-old woman who presented with back pain and progressive paraparesis secondary to a dorsal intramedullary arachnoid cyst detected on magnetic resonance imaging (MRI): the surgical planning and clinico-radiological outcome are discussed along with a review of the relevant literature.Study designCase report and literature review.Patient sampleOne patient affected by intramedullary arachnoid cyst.Outcome measuresMagnetic resonance imaging and pathological findings from operative specimens were used to confirm the diagnosis.MethodsA 31-year-old woman presented with a 7-year history of back pain that had worsened 3 months before admission to our department; for this reason, the patient had undergone a spinal MRI revealing the presence of a 1-cm cystic intramedullary lesion at the level T11–T12, with no contrast enhancement. After 2 months, the patient presented with a worsening of clinical symptoms complaining of severe back pain radiating to the lower extremities associated with a progressive paraparesis, urinary incontinence, and abdominal pain. Referred to our department, at the time of admission the patient was bedridden because of the impossibility of maintaining a standing position. The patient underwent a T11–T12 laminectomy with fenestration of the cyst.ResultsShe experienced an immediate relief of pain symptoms, and by the seventh postoperative day she was able to stand without help and walk a few meters with assistance. By the sixth postoperative month, the patient had significantly improved, having gained the ability to walk alone without assistance with complete resolution of the bladder dysfunctions, with no cyst recurrence after approximately 2 years of follow-up.ConclusionsIntramedullary arachnoid cysts should be considered in the differential diagnosis for intramedullary cystic lesions. A particular consideration deserves their occurrence in asymptomatic patients, who should be adequately informed on the possible natural evolution: when symptomatic, surgical therapy should be promptly offered, considering that a postoperative complete recovery is usually observed, regardless of the surgical technique.     

Cervical intradural extramedullary cavernoma presenting with isolated intramedullary hemorrhage

Among cavernomas of the central nervous system, spinal ones are rare. The true incidence of spinal cavernomas is unclear, but with widespread use of magnetic resonance imaging the number of cases is increasing. Furthermore, cavernomas represent only 5-12% of all vascular anomalies of the spinal cord, with a mere 3% reported to be intradural and intramedullary in location. Cervical spine intradural extramedullary cavernomas are very seldom seen, and only 4 cases have been reported in world literature previously. In this report, a unique case of an intradural extramedullary spinal cavernoma was surgically treated in a patient who presented only with an intramedullary hemorrhage.     

Advances in diagnosis and treatment of spinal hemangioblastomas

Spinal hemangioblastoma usually leads to large intramedullary cysts. Until recently clinical and conventional radiological examination was often not able to detect single or multiple tumor niduses at the wall of a secondary syringomyelia cyst. Thus during surgical exploration the tumor not infrequently was missed, leading to misinterpretation of the pathological entity as syringomyelia, spinal gliosis or low grade spinal astrocytoma. This often resulted in explorative biopsy or decompressive laminectomy. Clinical deterioration postoperatively was not uncommon due to the remaining tumor and increasing spinal cord enlargement. Now magnetic resonance imaging allows the exact preoperative localization of the medullary lesion apart from the accompanying cysts and facilitates differential diagnosis of primary syringomyelia or other spinal cord neoplasms. Microsurgical techniques and laser-assisted resection have improved the outcome. We report on eight patients with spinal hemangioblastoma treated in our clinic since the installation of magnetic resonance imaging and laser-assisted microneurosurgery.     

Spinal extradural arachnoid cyst

Background contextSpinal extradural arachnoid cysts are uncommon expanding lesions. Idiopathic arachnoid cysts are not associated with trauma or other inflammatory insults. If they enlarge, they usually present with progressive signs and symptoms of neural compression.PurposeTotal removal of the cyst and repair of the dural defect is the primary treatment for large thoracolumbar spinal extradural arachnoid cysts causing neurogenic claudication. Laminoplasty may prevent spinal deformities in long segmental involvement.Study designA clinical case was performed.Patient sampleWe report a case of 25-year-old man with 1-year history of progressive back pain radiating to both legs. His diagnosis was dorsal intraspinal extradural cystic lesion longing from the level of T11 to L2 on magnetic resonance imaging.Outcome MeasuresThe patient's pain levels were noted as he reported. Physiologic outcome was assessed on pre- and postoperative motor and sensory examination.MethodsThe patient underwent a T11–L2 laminotomy and radical cyst wall resection was performed. A small communication with the subarachnoid space was seen at the level of T12. It was sealed with tissue fibrinogen after repair with primary suture. Titanium miniplates were used for laminoplasty.ResultsFollow-up magnetic resonance imaging demonstrated cyst resolution, and neurologic examination revealed no sensory and motor deficit.ConclusionExtradural arachnoid cysts are primarily treated with total removal of the cyst wall and closure of the dural defect. Surgical treatment is curative for this rare lesion.