Multiple epidural lumbar chordomas without bone involvement in a 17-year-old female: a case report.

BACKGROUND CONTEXT: Chordomas are rare slow-growing neoplasms or malignant tumors that arise from embryonic rudiments of the primitive notochord. They account for 1% to 4% of all primary malignant bone tumors. Surgery in addition to radiotherapy is often used as the optimum treatment but with marginal effectiveness as these tumors are relatively radioresistant. To the best of our knowledge, there have been no reported cases of multiple and distinct epidural lesions without bone involvement in the literature. PURPOSE: To describe an unusual case of two separate epidural chordomas occurring in the lumbar spine without bone involvement. STUDY DESIGN: Case report. METHODS: Clinical, radiologic, and histopathologic evaluation of a 17-year-old female with two separate adjacent lumbar epidural chordomas without bone involvement treated with complete surgical excision is discussed. RESULTS: Four years after surgical resection of the tumors, the patient remains asymptomatic, and radiologic results revealed no recurrent lumbar epidural tumor. CONCLUSIONS: Epidural chordomas appear to have a better prognosis due, at least in part, to the ability to more easily completely excise them.

Tenosynovial giant cell tumor of the thigh: positron emission tomography findings.

Tenosynovial giant cell tumors (TGCTs) are pigmented villonodular proliferative lesions originating from the synovium, bursa, or joint. TGCTs tend to be locally aggressive, and there is a chance for multiple occurrences, which often lead to impairment of joint function. In this article, we report the case of a diffuse-type extra-articular TGCT found in the thigh of a 36-year-old woman. Surveillance F-18 fluorodeoxyglucose positron emission tomography detected increased activity within the left thigh. This activity was confirmed with magnetic resonance imaging and with surgical excision and histopathologic determination of the tumor. This patient's case suggests that TGCTs may be discovered and followed after resection with positron emission tomography.

The role of concurrent fusion to prevent spinal deformity after intramedullary spinal cord tumor excision in children.

OBJECTIVES: Spinal deformity is a common development after laminectomy and resection of pediatric intramedullary spinal cord tumors. Our objective is to compare the occurrence of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors that underwent decompression with fusion at the time of surgery to those that did not undergo fusion. METHODS: A retrospective chart review of 255 children with spinal cord tumors treated at 2 tertiary pediatric cancer centers between was performed. Of these, 52 patients with a biopsy-proven intramedullary spinal cord tumor had complete clinical records and radiographic data. Preoperative spinal alignment, surgical treatment, postoperative deformity, and risk factors for deformity were evaluated. All patients had at least 2-year follow-up. RESULTS: There were 18 females and 34 males with an average age of 8.1 ± 4.1 years. The average time to latest follow-up was 7.6 ± 5.3 years. Moderate or severe postresection spinal deformity (scoliosis > 25 degrees and/or sagittal plane abnormality > 20 degrees requiring bracing or surgery) developed in 57% (21/37) of resections without fusion (laminectomy or laminoplasty alone), and in 27% (4/15) of those with fusion (P = 0.05). Among skeletally mature children, 18 of 28 (64%) developed deformity after laminectomies and laminoplasties, compared with 22% (2/9) of the patients in the fusion group (P = 0.03). Removal of >3 lamina (P = 0.04) was associated with development of postoperative deformity. CONCLUSIONS: In the surgical treatment of patients with intramedullary spinal cord tumors, those that undergo instrumentation or in situ fusion at the time of spinal cord tumor excision are significantly less likely to develop postresection spinal deformity. LEVEL OF EVIDENCE: 3, Retrospective comparative study.

Efficacy of spinal instrumentation and fusion in the prevention of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors.

Postlaminectomy spinal deformity is a frequent problem after resection of a pediatric spinal cord tumor. However, the use of spinal fusion done at the time of resection in preventing the development of deformity is unknown. The purpose of our study was to assess the effectiveness of single-stage laminectomy, spinal cord decompression, and fusion for the prevention of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors. Clinical charts and radiographs of 33 children with spinal cord tumors were reviewed for treatment description, preoperative spinal alignment, postoperative spinal alignment, and subsequent medical and surgical treatment. Spinal deformity was graded by predefined criteria. Significant spinal deformity developed in 10 of 17 children with laminectomy only, 3 of 4 children with laminoplasty, and 3 of 12 children with spinal fusion (2/6 with instrumentation, 1/6 with in situ fusion). Excluding patients that progressed to paraplegia, 9 of 16 patients treated with resection without fusion compared with 1 of 10 patients treated with resection and fusion developed a spinal deformity (P = 0.04). Among skeletally mature children that did not progress to paraplegia, 9 of 15 treated with resection without fusion compared with 1 of 7 patients treated with resection and fusion developed a deformity (P = 0.05). Removal of greater than 4 laminae (P = 0.03) was found to be associated with the development of postresection spinal deformity.

Lumbar vertebral hemangioma presenting with the acute onset of neurological symptoms. Case report.

Vertebral hemangiomas are common entities that rarely present with neurological deficits. The authors report the unusual case of a large L-3 vertebral hemangioma with epidural extension in a 27-year-old woman who presented with hip flexor and quadriceps weakness, foot drop, and leg pain. The characteristics of the mass on magnetic resonance imaging suggested an aggressive, hypervascular lesion. The patient underwent embolization of the lesion followed by direct intralesional injection of ethanol. Significant resolution of clinical symptoms was observed immediately after the procedure and at her follow-up visits. Follow-up imaging studies obtained 9 months after the procedure also documented a considerable reduction in the size of the hemangioma with minimal loss of vertebral height and a mild kyphosis at the affected level. On repeated imaging studies obtained 21 months postoperatively, the size of the hemangioma and the degree of vertebral body compression were stable. As demonstrated in this case, patients with vertebral hemangiomas can present with acute nerve root compression and signs and symptoms similar to those of disc herniation. Vertebral hemangiomas can be treated effectively with interventional techniques such as embolization and ethanol injection.

Bow hunter's syndrome caused by accessory cervical ossification: posterolateral decompression and the use of intraoperative Doppler ultrasonography.

BACKGROUND: Bow hunter's syndrome refers to symptomatic vertebrobasilar insufficiency provoked by physiologic head rotation. CASE DESCRIPTION: We report a unique case of bow hunter's syndrome caused by an accessory cervical ossification and the first use of intraoperative Doppler ultrasonography directly upon the vertebral artery during the surgical repair. After a traumatic motor-vehicle collision, the patient developed recurrent syncopal episodes when he turned his head abruptly to the right. Transcranial Doppler studies and vertebral angiography with the patient's neck rotated into the symptomatic position revealed marked reduction of vertebral artery flow, and fine-cut CT of the upper cervical spine demonstrated the compressive accessory ossicle. Intraoperative Doppler ultrasound performed with the head in neutral and rotated positions, before and after surgical decompression, demonstrated restoration of blood flow in the vertebral artery. We discuss the mechanisms of bow hunter's syndrome and the advantages of intraoperative Doppler ultrasonography. CONCLUSION: This case describes the first use of intraoperative Doppler ultrasonography directly upon the vertebral artery to provide an unrestricted real-time assessment of the surgical decompression for bow hunter's syndrome.

Intracranial capillary hemangioma: case report and review of the literature.

BACKGROUND: Capillary hemangiomas are benign vascular lesions that commonly present at birth or in early infancy on the face, scalp, back, or chest. The authors present an exceedingly rare case of an intracranial capillary hemangioma arising in an adult. Only 4 biopsy-proven cases have been reported in the pediatric population previous to this case report. CASE DESCRIPTION: A 31-year-old pregnant woman presented at 38 weeks of gestation with severe headaches, nausea, and vomiting. Imaging revealed an extra-axial mass lesion arising from the tentorium with both supra- and infratentorial components. The patient underwent a resection of her tumor, which was diagnosed as a capillary hemangioma by histopathologic examination. The patient required 2 further resections after the lesion exhibited a rapid regrowth from residual tumor in the left transverse sinus. The patient has remained free of disease 41 months out from her third surgery. CONCLUSIONS: Intracranial capillary hemangiomas are exceedingly rare entities, with a capability for rapid growth. When gross total resection cannot be achieved, these patients should be observed closely, and the use of adjuvant radiotherapy should be considered.

Error analysis of MRI and leksell stereotactic frame target localization in deep brain stimulation surgery.

Stereotactic deep brain stimulation (DBS) is the surgical treatment of choice for medication-refractory patients with Parkinson's disease and essential tremor. The subthalamic nucleus and ventral intermediate nucleus of the thalamus appear to be effective targets for electrode placement. Because these targets are small and encased in fiber tracts, their localization can be exceedingly difficult. However, the precision of electrode placement is crucial for obtaining successful results. Currently, surgeons rely on preoperative MRI or CT images to derive stereotactic coordinates for targeting sites such as the subthalamic nucleus and ventral intermediate nucleus of the thalamus coupled with microelectrode recordings during surgery for proper electrode placement. However, it has been argued that the stereotactic head frame produces detrimental artifacts during MRI. We examined MRI images taken from 11 patients undergoing repeat DBS surgery, and determined the coordinates of the previously placed electrode. We then set the Leksell G stereotactic frame to these coordinates and obtained fluoroscope-localizing images. Using MATLAB image analysis tools, we were able to quantify the 3-dimensional error in target localization by measuring the distance from the electrode tip to the targeted coordinate. The mean errors were 0.09 +/- 0.34 mm perpendicular, lateral to medial, 0.01 +/- 0.32 perpendicular, posterior to anterior, and -0.08 +/- 0.33 mm parallel to the electrode, superior to inferior. According to statistical analysis, the error was random and did not seem to move in any predictable fashion. Therefore, we conclude that preoperative MRI images can be safely used in DBS surgery, and they do not negatively affect its accuracy.

Management of patients with schwannomatosis: report of six cases and review of the literature.

BACKGROUND: Schwannomatosis is a rare tumor syndrome characterized by the presence of multiple schwannomas without the stigmata of neurofibromatosis (NF) Type 1 or 2. To better understand the natural history and clinical management of the syndrome, a retrospective review was conducted of patients diagnosed with schwannomatosis over an 11-year period at the University of Pennsylvania Medical Center (UPMC). METHODS: Between 1990 and 2001, 131 patients underwent surgery for resection of spinal or peripheral nerve schwannomas in the Department of Neurosurgery at the University of Pennsylvania Medical Center. Among the 131 patients, there were 6 who had two or more pathologically proven schwannomas without radiographic or clinical evidence of vestibular schwannomas. The hospital charts, clinic notes, radiology films, operative reports, pathology slides, and reports from all 6 patients were retrospectively reviewed. RESULTS: The patient population consisted of 6 patients with a mean age of 48.7 (3 male: 3 female). All patients had enhanced brain magnetic resonance imaging (MRI) scans that were negative for vestibular schwannomas. Ophthalmological and general physical examinations did not reveal any findings suggestive of NF. There was no family history of NF or schwannomatosis. The locations of the schwannomas included intraspinal (multiple sites), paraspinal, brachial plexus, femoral nerve, sciatic nerve, calf, forearm, retroperitoneum, and middle cranial/infratemporal fossa region. The common presenting symptoms included paresthesias, palpable mass, pain, or weakness. All 6 patients underwent surgical resection of symptomatic lesions. CONCLUSIONS: For patients with schwannomatosis, surgery is indicated for symptomatic lesions, while asymptomatic tumors are followed conservatively. Because these patients are at increased risk for developing multiple schwannomas, we recommend regular surveillance and offer genetic counseling even though the pattern of inheritance is unknown.

Intradural venous varix: a rare cause of an intradural lumbar spine lesion.

STUDY DESIGN: A case of intradural venous varix producing a mass-like lesion in the lumbar spine is reported. OBJECTIVE: To present a rare cause of an intradural mass lesion along the cauda equina. To present radiologic findings of this entity and to offer a hypothesis for its formation. SUMMARY OF BACKGROUND DATA: Epidural venous varices have been described previously in the literature. To the authors' knowledge, no cases of intradural venous varix presenting as an intradural mass have been reported previously. MATERIALS AND METHODS: An elongated, serpentine intradural mass was discovered on routine lumbar MRI performed for investigation of the patient's radiculopathy. The patient underwent laminectomy and intradural exploration. RESULT: On surgical exploration, a serpentine, massively dilated vein was identified, which distorted the normal nerve roots. CONCLUSION: A venous varix should be considered in the differential diagnosis of an intradural lesion in the lumbar spine in the appropriate clinical setting.

Complications of invasive monitoring used in intractable pediatric epilepsy.

Invasive monitoring for intractable epilepsy is useful when the epileptogenic focus is in question even after an extensive noninvasive presurgical evaluation, or when the epileptogenic focus is located in or near eloquent cortex. From June 1989 to June 2001 at the Children's Hospital of Philadelphia, 64 children with intractable epilepsy underwent 67 invasive monitoring procedures as part of their presurgical evaluation. The average age at implantation was 10 years. In all but two cases, subdural strips and grids were used. Depth electrodes, when used, were placed stereotactically or under direct vision. The average duration of the monitoring period was 5.87 days. Every patient had intradural cultures sent during removal of the electrodes, and lumbar punctures were performed in 15 patients. Twenty-one patients had at least one episode of a CSF leak. Of the 67 patients, 10 had positive intradural cultures, only 1 of whom had a positive lumbar puncture and none of whom developed clinically significant meningitis. No clinically relevant hemorrhages occurred as a result of the invasive monitoring. One patient did have a transient visual field loss after placement of an occipital grid. While CSF leaks are common after invasive monitoring despite precautions, clinically significant CSF infections are uncommon and appear to be unrelated to the duration of monitoring, the occurrence of a CSF leak or the length of time the patient is on perioperative antibiotics. We conclude that invasive monitoring for intractable epilepsy is generally safe.

Epidural hematoma as a complication of sphenoid sinusitis and epidural abscess: a case report and literature review.

Imaging is of paramount importance in early diagnosis of epidural abscess and its intracranial complications. Typical CT imaging features of an epidural abscess include a hypodense lentiform extra-axial collection with rim enhancement. We present a case of epidural abscess that was hyperdense on CT scan due to the presence of associated epidural hematoma. The literature is reviewed regarding this unusual complication of epidural abscess.

Recovery of language after left hemispherectomy in a sixteen-year-old girl with late-onset seizures.

Hemispherectomy is a very effective surgical treatment for intractable seizures that occur in the setting of Rasmussen's syndrome. The decision of when to perform a dominant hemispherectomy depends greatly on how late the surgeon believes some shift in language to the nondominant hemisphere can occur. We report a right-handed patient with Rasmussen's syndrome who underwent a left hemispherectomy at the age of 16 and has had excellent control of her seizures and remarkable language recovery. Our results indicate that dominant hemispherectomy in adolescence in the setting of late-onset seizures and Rasmussen's syndrome in the profoundly aphasic patient does not commit the patient to the prognosis of a fixed language deficit.

Intramedullary injection of contrast into the cervical spinal cord during cervical myelography: a case report.

STUDY DESIGN: A case of iatrogenic intramedullary contrast injection during a C1-C2 cervical myelography is reported. OBJECTIVE: To investigate the occurrence of iatrogenic intramedullary contrast injection during a current C1-C2 cervical myelography. SUMMARY OF BACKGROUND DATA: Intramedullary injection of contrast is a rare but serious complication of C1-C2 cervical myelography that has not been reported since the widespread use of magnetic resonance imaging and the NASCIS III study protocol. METHODS: A 39-year-old woman received an iatrogenic intramedullary contrast injection during a C1-C2 cervical myelography. RESULTS: During the procedure the patient reported right-side face, neck, and arm pain and parethesias. After the procedure, right arm weakness and diffuse hyperreflexia developed. Postmyelography imaging demonstrated intramedullary contrast and cord swelling. High-dose methylprednisolone was administered intravenously and the patient's symptoms improved. The literature and management of this rare complication are reviewed. CONCLUSIONS: Intramedullary cord injection is a rare complication of cervical myelography. The mechanism of spinal cord injury appears to involve a combination of physical compression from the injected liquid and neurotoxicity of the contrast material. Iohexol rather than metrizamide should be used when C1-C2 myelography is indicated in patients who are unable to undergo magnetic resonance imaging, or those whose pathology is inadequately demonstrated magnetic resonance imaging alone. In the event of contrast injection into the spinal cord, administration of high-dose methylprednisolone is recommended.