Coupling Hematoma Evacuation with Immune Profiling for Analysis of Neuroinflammation After Primary Intracerebral Hemorrhage: A Pilot Study.

OBJECTIVE: We sought to explore the use and feasibility of an integrated hematoma evacuation/tissue preservation system coupled with immune profiling to assess human ex vivo immune cell populations from brain hematoma samples after intracerebral hemorrhage (ICH). METHODS: In this nonrandomized, noncontrolled pilot/feasibility study of 7 patients with primary supratentorial ICH, a hematoma evacuation device and integrated tissue preservation system were used to obtain hematoma samples during surgical evacuation. Samples were processed, cryopreserved, and analyzed using flow cytometry to determine the relative distribution of immune cell populations compared with peripheral blood mononuclear cells from healthy control subjects. RESULTS: This study demonstrates proof of concept for an integrated hematoma evacuation and sample preservation system to collect human brain hematoma samples for flow cytometry analysis after acute human ICH. In our preliminary analysis, hematoma samples demonstrated a different makeup of white blood cells than peripheral blood from healthy controls. CONCLUSIONS: Flow cytometry analysis of hematoma samples in ICH demonstrates the potential to provide important insights into neuroinflammation associated with ICH.

Two-Level Minimally Invasive Lumbar Laminectomy and Foraminotomy: 2-Dimensional Operative Video.

Minimally invasive lumbar laminectomies utilize tubular retractors to minimize tissue disturbance, blood loss, and hospital length of stay compared to open operations. Here, we highlight the operative treatment of a 68-yr-old male entailing a 2-level minimally invasive lumbar laminectomy and foraminotomy at L2-L3 and L3-L4. The patient initially presented with symptoms of treatment-refractory lower extremity numbness and limited ambulation. His imaging demonstrated coronal scoliosis and severe lumbar central and foraminal stenosis at L2-L3 and L3-L4, with enlarged spinous processes, laminae, and facets. The patient consented to the procedure and publication of their image. The operation proceeded with the patient in a prone position with paramedian dissection to the lamina through a minimally invasive tubular retractor. Laminectomies and foraminotomies were performed at each level with high-speed drill and a Kerrison rongeur, with care to identify and protect the relevant spinal nerve roots. Postoperatively, the patient reported significantly reduced numbness and improved ambulation, with a well-healed surgical incision notably smaller than those produced in an open operation.

Glioblastoma in the elderly: the effect of aggressive and modern therapies on survival.

OBJECTIVE: The prognosis of elderly patients with glioblastoma (GBM) is universally poor. Currently, few studies have examined postoperative outcomes and the effects of various modern therapies such as bevacizumab on survival in this patient population. In this study, the authors evaluated the effects of various factors on overall survival in a cohort of elderly patients with newly diagnosed GBM. METHODS: A retrospective review was performed of elderly patients (≥ 65 years old) with newly diagnosed GBM treated between 2004 and 2010. Various characteristics were evaluated in univariate and multivariate stepwise models to examine their effects on complication risk and overall survival. RESULTS: A total of 120 patients were included in the study. The median age was 71 years, and sex was distributed evenly. Patients had a median Karnofsky Performance Scale (KPS) score of 80 and a median of 2 neurological symptoms on presentation. The majority (53.3%) of the patients did not have any comorbidities. Tumors most frequently (43.3%) involved the temporal lobe, followed by the parietal (35.8%), frontal (32.5%), and occipital (15.8%) regions. The majority (57.5%) of the tumors involved eloquent structures. The median tumor size was 4.3 cm. Every patient underwent resection, and 63.3% underwent gross-total resection (GTR). The vast majority (97.3%) of the patients received the postoperative standard of care consisting of radiotherapy with concurrent temozolomide. The majority (59.3%) of patients received additional agents, most commonly consisting of bevacizumab (38.9%). The median survival for all patients was 12.0 months; 26.7% of patients experienced long-term (≥ 2-year) survival. The extent of resection was seen to significantly affect overall survival; patients who underwent GTR had a median survival of 14.1 months, whereas those who underwent subtotal resection had a survival of 9.6 months (p = 0.038). Examination of chemotherapeutic effects revealed that the use of bevacizumab compared with no bevacizumab (20.1 vs 7.9 months, respectively; p < 0.0001) and irinotecan compared with no irinotecan (18.0 vs 9.7 months, respectively; p = 0.027) significantly improved survival. Multivariate stepwise analysis revealed that older age (hazard ratio [HR] 1.06 [95% CI1.02-1.10]; p = 0.0077), a higher KPS score (HR 0.97 [95% CI 0.95-0.99]; p = 0.0082), and the use of bevacizumab (HR 0.51 [95% CI 0.31-0.83]; p = 0.0067) to be significantly associated with survival. CONCLUSION: This study has demonstrated that GTR confers a modest survival benefit on elderly patients with GBM, suggesting that safe maximal resection is warranted. In addition, bevacizumab significantly increased the overall survival of these elderly patients with GBM; older age and preoperative KPS score also were significant prognostic factors. Although elderly patients with GBM have a poor prognosis, they may experience enhanced survival after the administration of the standard of care and the use of additional chemotherapeutics such as bevacizumab.

Initial experience with extreme angle cervical screw for treatment of trauma and cervical spondylosis.

In this study, we have described our initial experience and surgical technique of extreme angle screw placement in the cervical and upper thoracic spine of a cohort of patients undergoing posterior fusion. This extreme angle screw facilitates rod placement without need for any coronal contouring of the rod or offset connectors despite the varied entry site locations for posterior instrumentation and the different trajectories and pathways of these screws. From ruary 2011 to July 2011, extreme angle screws were placed in twenty consecutive adult patients who underwent posterior cervical, occipital-cervical or cervical-thoracic fusions. The primary diagnosis was cervical spondylotic myelopathy (13), trauma (4), and pseudoarthrosis with stenosis (3). Eight patients had gross instability. A total of 196 screws were placed; half of the cases involved instrumentation at or within the C3-7 segments (10) and the others included constructs extending to occipital bone, C2, T1, or T2 (10). Of all twenty cases, there were no perioperative hardware complications. At long-term follow-up, two patients required reoperation, one for hardware failure and the other for single level symptomatic pseudoarthrosis. We conclude that extreme angle screw use in the posterior cervical spine provides an evolution in posterior instrumentation that maximizes the biomechanical strength of a construct, allows for easy rod placement, and may improve the restoration of sagittal alignment. Overall, extreme angle screws facilitate rod placement even for screws offset from the natural plane of the rod, thereby avoiding the need for coronal contouring or placement of offset connectors.

Insurance disparities in the outcomes of spinal cord stimulation surgery.

OBJECTIVES: The Affordable Care Act aims to expand health insurance and to help narrow existing health care disparities. Medicaid patients have previously been noted to be at an increased risk for impaired access to health care, delayed medical treatment, and the receipt of substandard care. Conversely, those with commercial insurance may be subject to overtreatment. The goal of this study was to evaluate how Medicaid versus commercial insurance status affects outcomes following spinal cord stimulation (SCS) surgery. MATERIALS AND METHODS: A retrospective cohort study of 13,774 patients underwent either percutaneous or paddle permanent SCS implantation, selected from the Thomson Reuter's MarketScan database between 2000 and 2009. Patients were characterized by age at initial procedure, gender, baseline comorbidity burden, procedure-associated diagnosis code, follow-up, and type of insurance (Medicaid vs. commercial insurance). Outcome measures included probability of reoperation, timing and type of reoperation, presence of postoperative complications (immediate, 30 days, and 90 days), and overall utilization of health resources postoperatively. Multivariate analysis was performed comparing the relative effect of insurance status on outcomes following initial surgery. RESULTS: Medicaid patients had greater healthcare resource utilization as measured by medications prescribed, emergency department visits, and length of stay; however, commercially insured patients had significantly higher overall costs ($110,908 vs. $64,644, p < 0.0001). Commercial and Medicaid patients did not significantly differ in their complication rates during the index hospitalization or at 30 days or 90 days postoperatively. The group were also not significantly different in their two-year reoperation rates (7.32% vs. 5.06%, p = 0.0513). CONCLUSIONS: There are substantial insurance disparities that affect healthcare utilization and overall cost following SCS. Efforts for national healthcare reform should examine system factors that will reduce socioeconomic disparities in outcomes following SCS.

The history of the combined supra- and infratentorial approach to the petroclival region.

Lesions of the ventrolateral brainstem, clivus, and cerebellopontine angle pose significant challenges for surgeons, and the rate of morbidity and mortality from classic neurosurgical approaches has proven to be unacceptably high. Early attempts to expose this region consisted primarily of an extended suboccipital craniectomy, with opening of the tentorium and ligation of the sigmoid sinus for additional exposure. During the 1960s, technological innovations including the surgical microscope and the pneumatic drill allowed surgeons to gain additional exposure by removing more bone from the base of the skull. This let surgeons define combined infra- and supratentorial approaches, which rely less on brain retraction to resect these difficult tumors successfully. These approaches rely on a combined posterior mastoid approach with an anterior petrosectomy. The evolution of this approach is discussed in this paper.

Comparison of superior-level facet joint violations during open and percutaneous pedicle screw placement.

BACKGROUND: Superior-level facet joint violation by pedicle screws may result in increased stress to the level above the instrumentation and may contribute to adjacent segment disease. Previous studies have evaluated facet joint violations in open or percutaneous screw cases, but there are no reports describing a direct institutional comparison. OBJECTIVE: To compare the incidence of superior-level facet violation for open vs percutaneous pedicle screws and to evaluate patient and surgical factors that affect this outcome. METHODS: We reviewed 279 consecutive patients who underwent an index instrumented lumbar fusion from 2007 to 2011 for degenerative spine disease with stenosis with or without spondylolisthesis. We used a computed tomography grading system that represents progressively increasing grades of facet joint violation. Patient and surgical factors were evaluated to determine their impact on facet violation. RESULTS: Our cohort consisted of 126 open and 153 percutaneous cases. Percutaneous procedures had a higher overall violation grade (P = .02) and a greater incidence of high-grade violations (P = .006) compared with open procedures. Bivariate analysis showed significantly greater violations in percutaneous cases for age < 65 years, obesity, pedicle screws at L4, and 1- and 2-level surgeries. Multivariate analysis showed the percutaneous approach and depth of the spine to be independent risk factors for high-grade violations. CONCLUSION: This study demonstrates greater facet violations for percutaneously placed pedicle screws compared with open screws.

Microsurgical management of hypoglossal schwannomas over 3 decades: a modified grading scale to guide surgical approach.

BACKGROUND: Schwannomas originating from the hypoglossal nerve are extremely rare. Microsurgical resection with the goal for cure has traditionally been associated with a high risk of postoperative deficits. OBJECTIVE: To summarize our clinical experience using tailored cranial base approaches for these formidable lesions. METHODS: The clinical records of 13 patients were retrospectively reviewed. In addition, all reported patients in the literature were reviewed. The extreme lateral infrajugular transcondylar-transtubercular exposure approach was used in all of our patients. Based on our experience and literature analysis, we propose the following modified grading scale to facilitate surgical planning: type A, intradural tumors; type B, dumbbell-shaped tumors; type C, extracranial tumors; and type D, peripheral tumors. RESULTS: All 13 patients underwent total, near-total, or subtotal tumor resection. Eight patients were men, 5 were women (mean age, 41.7 years). Sural nerve graft reconstruction for the hypoglossal nerve was performed in 4 patients. Three of the 4 patients in whom nerve reconstruction was performed regained satisfactory movement of their tongue. In the review of the literature, the mean patient age was 45.8 years. Patients presented with tongue atrophy (91.6%), headache (60.9%), and dysphagia (31.8%). The tumors were categorized as type A in 31.7% of these patients, type B in 38.6%, type C in 6.2%, and type D in 23.4%. CONCLUSION: The extreme lateral infrajugular transcondylar-transtubercular exposure approach, which is a modification of the extreme lateral suboccipital approach, provides sufficient exposure for most intracranial dumbbell-shaped hypoglossal schwannomas. Hypoglossal nerve reconstruction using a sural nerve graft improves tongue atrophy and movement for patients with resected nerves.

Minimally invasive lumbar interbody fusion in patients older than 70 years of age: analysis of peri- and postoperative complications.

BACKGROUND: The number of spine operations performed in the elderly population is rising. OBJECTIVE: To identify and describe perioperative and postoperative complications in patients 70 years and older who have undergone minimally invasive lumbar interbody spine fusion. METHODS: A retrospective analysis was performed on 66 consecutive patients aged 70 years or older who underwent a minimally invasive interbody lumbar fusion. Electronic medical records were analyzed for patient demographics, procedures, and perioperative and postoperative complications. RESULTS: Between 2000 and 2009, 66 patients with an average age of 74.9 years (range, 70-86 years) underwent 68 lumbar interbody fusions procedures. The mean follow-up was 14.7 months (range, 1.5-50 months). The minimally invasive approaches included 41 cases of extreme lateral interbody fusion and 27 minimally invasive transforaminal lumbar interbody fusions. We observed 5 major (7.4%) and 17 minor (25%) complications. The 5 major complications consisted of 4 cases of interbody graft subsidence and 1 adjacent level disease. There were no intraoperative medical complications. There were no myocardial infarctions, pulmonary embolisms, hardware complications requiring removal, wound infections, major visceral, vascular, neural injuries, or death in the study period. CONCLUSION: Minimally invasive interbody fusions can be performed in the elderly (ages 70 years and older) with an overall low rate of major complications. Graft subsidence in this population when not supplemented with posterior instrumentation is a concern. Age should not be a deterrent to performing complex minimally invasive interbody fusions in the elderly.

Unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst.

The authors describe a rare case of unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. Three similar cases have been reported with pathological classification still under consideration. One case was classified as an intraneural ganglion cyst and 2 cases were classified as atlantooccipital joint synovial cysts.

Response of malignant scalp dermatofibrosarcoma to presurgical targeted growth factor inhibition.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, malignant cutaneous tumor that sparingly presents on the scalp. Dermatofibrosarcomas often result from the formation of a fusion oncogene on translocated or supernumerary ring chromosomes 17 and 22, causing the overexpression of PDGFRbeta driven by the COL1A1 promoter. Because of uncertainty surrounding appropriate treatment of aggressive scalp DFSP, the authors performed an extensive review of the available data from a MEDLINE (Ovid) search to describe the clinical presentation and treatment options for this rare tumor. Their search identified 39 different cases, including the illustrative case presented in this study. Adjuvant therapy for this malignant lesion is not universally established in the literature. In the present case, the authors successfully treated a locally invasive scalp DFSP with presurgical therapy that specifically inhibited the PDGFbeta receptor. Imatinib significantly shrank the DFSP tumor mass, reduced hypervascularity, reduced metabolic activity on PET scanning, and permitted a safe gross-total resection. Although wide excision and Mohs micrographic surgery remain the standard surgical treatments for DFSP, the authors illustrate that presurgical chemotherapeutic treatment by imatinib provides a critical adjunct to traditional therapy.

Cholesterol granuloma of the lateral ventricle. Case report.

Cholesterol granulomas (CGs) are benign lesions resulting from an inflammatory reaction to cholesterol and hemosiderin. These masses most often arise within the temporal bone or nasal sinuses; intracerebral CGs are extremely rare. In this report the authors present an unusual case of a CG arising within the lateral ventricle. The patient presented with transient hemiparesis and numbness. Computed tomography and magnetic resonance imaging demonstrated a cystic partially enhancing midline mass within the right lateral ventricle, expanding the ventricle and displacing the septum pellucidum. The patient underwent an interhemispheric, transcallosal resection of the lesion. Microscopic examination revealed a granulomatous inflammatory lesion containing cholesterol clefts, macrophages, and hemosiderin. Embedded within the granulomatous response were foci of tiny cystlike structures lined by nonciliated flattened cuboidal epithelium, consistent with the diagnosis of CG. To the authors' knowledge this is the first reported case of CG presenting as an intraventricular mass. The origin of this lesion is unclear, but it may relate to prior traumatic brain injury. The authors describe the presentation, imaging findings, histopathological characteristics, and surgical treatment of this rare lesion and related pathological entities.

Systemic CTLA-4 blockade ameliorates glioma-induced changes to the CD4+ T cell compartment without affecting regulatory T-cell function.

PURPOSE: Patients with malignant glioma suffer global compromise of their cellular immunity, characterized by dramatic reductions in CD4(+) T cell numbers and function. We have previously shown that increased regulatory T cell (T(reg)) fractions in these patients explain T-cell functional deficits. Our murine glioma model recapitulates these findings. Here, we investigate the effects of systemic CTLA-4 blockade in this model. EXPERIMENTAL DESIGN: A monoclonal antibody (9H10) to CTLA-4 was employed against well-established glioma. Survival and risks for experimental allergic encephalomyelitis were assessed, as were CD4(+) T cell numbers and function in the peripheral blood, spleen, and cervical lymph nodes. The specific capacities for anti-CTLA-4 to modify the functions of regulatory versus CD4(+)CD25(-) responder T cells were evaluated. RESULTS: CTLA-4 blockade confers long-term survival in 80% of treated mice, without eliciting experimental allergic encephalomyelitis. Changes to the CD4 compartment were reversed, as anti-CTLA-4 reestablishes normal CD4 counts and abrogates increases in CD4(+)CD25(+)Foxp3(+)GITR(+) regulatory T cell fraction observed in tumor-bearing mice. CD4(+) T-cell proliferative capacity is restored and the cervical lymph node antitumor response is enhanced. Treatment benefits are bestowed exclusively on the CD4(+)CD25(-) T cell population and not T(regs), as CD4(+)CD25(-) T cells from treated mice show improved proliferative responses and resistance to T(reg)-mediated suppression, whereas T(regs) from the same mice remain anergic and exhibit no restriction of their suppressive capacity. CONCLUSIONS: CTLA-4 blockade is a rational means of reversing glioma-induced changes to the CD4 compartment and enhancing antitumor immunity. These benefits were attained through the conferment of resistance to T(reg)-mediated suppression, and not through direct effects on T(regs).

Ventriculoperitoneal shunt failure causing myelopathy in a patient with bilateral jugular vein occlusion. Case report.

The authors describe the case of a 36-year-old woman with bilateral internal jugular vein occlusion, hydrocephalus, and Dandy-Walker variant who presented with myelopathy that was ultimately attributed to ventriculoperitoneal (VP) shunt failure. Computed tomography (CT) angiography of the head and neck revealed epidural venous engorgement within the cervical spine, greater that 50% narrowing of the C2-5 spinal canal, and compression of the cervical spinal cord. After successful shunt revision, postoperative CT angiography revealed decreased venous engorgement as well as decompression of the cervical spinal cord, and the patient's myelopathy improved. This case represents a fascinating clinical presentation of VP shunt failure, highlighting the physiological importance of the external jugular pathways involved in cerebral venous drainage.

Genetic analysis of intracranial tumors in a murine model of glioma demonstrate a shift in gene expression in response to host immunity.

For the study of malignant glioma, we have previously characterized a highly tumorigenic murine astrocytoma, SMA-560, which arose spontaneously in an inbred, immunocompetent VM/Dk mouse. Using this cell line as a model of murine glioma, we performed DNA microarray analysis of autologous normal murine astroctyes (NMA) and SMA-560 tumor cells grown in monolayer culture or intracranially in syngeneic immunocompetent or immunocompromised hosts in order to determine whether tumors grown in vitro recreate the complex genetic regulation that occurs in vivo. Our findings support our hypothesis that glioma phenotype in vitro may be quite different in vivo and significantly altered by in situ growth factors and other invading cell populations.

Systemic anti-CD25 monoclonal antibody administration safely enhances immunity in murine glioma without eliminating regulatory T cells.

PURPOSE: Elevated proportions of regulatory T cells (T(reg)) are present in patients with a variety of cancers, including malignant glioma, yet recapitulative murine models are wanting. We therefore examined T(regs) in mice bearing malignant glioma and evaluated anti-CD25 as an immunotherapeutic adjunct. EXPERIMENTAL DESIGN: CD4+CD25+Foxp3+GITR+ T(regs) were quantified in the peripheral blood, spleens, cervical lymph nodes, and bone marrow of mice bearing malignant glioma. The capacities for systemic anti-CD25 therapy to deplete T(regs), enhance lymphocyte function, and generate antiglioma CTL responses were assessed. Lastly, survival and experimental allergic encephalitis risks were evaluated when anti-CD25 was combined with a dendritic cell-based immunization targeting shared tumor and central nervous system antigens. RESULTS: Similar to patients with malignant glioma, glioma-bearing mice show a CD4 lymphopenia. Additionally, CD4+CD25+Foxp3+GITR+ T(regs) represent an increased fraction of the remaining peripheral blood CD4+ T cells, despite themselves being reduced in number. Similar trends are observed in cervical lymph node and spleen, but not in bone marrow. Systemic anti-CD25 administration hinders detection of CD25+ cells but fails to completely eliminate T(regs), reducing their number only moderately, yet eliminating their suppressive function. This elimination of T(reg) function permits enhanced lymphocyte proliferative and IFN-gamma responses and up to 80% specific lysis of glioma cell targets in vitro. When combined with dendritic cell immunization, anti-CD25 elicits tumor rejection in 100% of challenged mice without precipitating experimental allergic encephalitis. CONCLUSIONS: Systemic anti-CD25 administration does not entirely eliminate T(regs) but does prevent T(reg) function. This leads to safe enhancement of tumor immunity in a murine glioma model that recapitulates the tumor-induced changes to the CD4 and T(reg) compartments seen in patients with malignant glioma.

Microvascular decompression for glossopharyngeal neuralgia: long-term effectiveness and complication avoidance.

OBJECTIVE: To establish the long-term safety, efficacy, and durability of microvascular decompression (MVD) for the treatment of glossopharyngeal neuralgia, this study presents the immediate (<6 mo) postoperative and long-term results of a large series of 47 patients with treated with MVD. METHODS: Operative reports and hospital charts were analyzed to collect demographic information, clinical presentation, and surgical findings. Surgical results and complications were ascertained by direct patient contact or by contact with the patient's family or physician if the patient was dead. Long-term (>10 yr) personal follow-up was available for 29 of 47 patients. RESULTS: Forty-six (98%) of 47 patients experienced complete relief of pain immediately after MVD. Long-term follow-up was available for 29 of these 47 patients (range, 125-211 mo; median, 152 mo, or 12.7 yr), and 28 of these 29 patients continued to be pain-free. Permanent neurological deficits (>6 mo) attributed to the surgery were observed in 5 (11%) of 47 patients. Of these patients, 4 of 5 had mild hoarseness or dysphagia or both, and one had a Grade II/VI facial nerve paresis. CONCLUSION: This study demonstrates that MVD is a safe, effective, and durable surgical procedure for producing prolonged pain relief in patients with medically intractable glossopharyngeal neuralgia.

Efficacy of intracerebral microinfusion of trastuzumab in an athymic rat model of intracerebral metastatic breast cancer.

PURPOSE: The monoclonal antibody (MAb) trastuzumab (Herceptin) effectively treats HER2-overexpressing extracerebral breast neoplasms. Delivery of such macromolecule therapeutic agents to intracerebral metastases, however, is limited by the tight junctions characteristic of the cerebral vasculature. Direct intracerebral microinfusion (ICM) is a technique that bypasses this blood-brain barrier and allows for a greater delivery of drugs directly into intracerebral tumors. EXPERIMENTAL DESIGN: A human breast cancer cell line transfected to overexpress HER2, MCF-7/HER2-18, was transplanted into the cerebrum of athymic rats. Saline, trastuzumab, or an isotype-matched control MAb was delivered systemically or by ICM to assess toxicity and efficacy. RESULTS: No clinical or histological toxicity related to trastuzumab was evident under any of the conditions studied. Delivery of trastuzumab (2 mg/kg) i.p. led to a median survival of 26.5 days, whereas treatment with trastuzumab (2 mg/kg) by ICM increased the median survival by 96% to 52 days, with two of nine rats surviving >120 days (P = 0.009). Treatment with an isotype-matched control MAb (16 mg/kg) resulted in a median survival of 21 days, which did not differ significantly from the survival of rats treated by ICM with saline (16 days; P = 0.42). Treatment by ICM with trastuzumab (16 mg/kg) led to a median survival of 45 days, with 2 of 10 rats surviving >120 days. These results represent 181% and 114% increases in median survival over the saline and MAb controls, respectively (P < 0.001). CONCLUSION: ICM of trastuzumab is safe and superior to systemic delivery as therapy for HER2-overexpressing intracerebral neoplasms in an athymic rat model.

Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report.

OBJECTIVE AND IMPORTANCE: Spinal cord involvement in Klippel-Trenaunay-Weber (KTW) syndrome is rare. Cases of intradural spinal cord arteriovenous malformations (AVMs) have been associated with this syndrome. Likewise, cases of epidural hemangioma and angiomyolipoma have been reported to occur at the same segmental level as cutaneous hemangioma in KTW syndrome. This report details a rare case of an extradural thoracic AVM in a patient with KTW syndrome. CLINICAL PRESENTATION: A 30-year-old man presented with a 10-month history of progressive myelopathy, bilateral lower-extremity weakness, and numbness, with the right side affected more than the left. His symptoms had progressed to the point that he was unable to walk. The patient had the characteristic manifestations of KTW syndrome, including numerous cutaneous angiomas and cavernomas, limb hypertrophy and syndactyly, and limb venous malformations. A magnetic resonance imaging scan and subsequent angiogram demonstrated a large extradural AVM causing cord compression at the T3-T4 levels. INTERVENTION: The patient underwent two separate endovascular procedures, including embolization of upper thoracic and thyrocervical trunk feeders. Subsequently, he underwent T1-T4 laminectomy and microsurgical excision of the AVM. Clinically, the patient improved such that he could walk without assistance. CONCLUSION: KTW syndrome represents a spectrum of clinical presentations. Although involvement of the spinal cord is uncommon, the manifestations of this syndrome may include both intradural and extradural AVMs in addition to various tumors.