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## Bilateral frontal intracranial xanthoma associated with type II hyperlipidemia in a 42-year-old woman: illustrative case

### BACKGROUND

Xanthoma is a granulomatous lesion that develops from leakage of circulating serum lipoprotein into the surrounding tissue. An isolated intracranial xanthoma is rarely reported and usually misdiagnosed. Intracranial xanthoma is also rarely found in patients with hyperlipidemia. To the best of the authors’ knowledge, no previous studies and literature have reported bilateral involvement of intracranial xanthoma in the frontal lobe.

### OBSERVATIONS

The authors reported an unusual case of bilateral involvement of intracranial xanthoma in the frontal lobe with associated type II hyperlipidemia in a 42-year-old woman. Macroscopically, the tumor had an appearance of greyish-yellow color with a brittle, solid consistency. Histopathological examination revealed numerous lipid-laden macrophages surrounded by a cystic, necrotic, partially hemorrhagic area, with some parts consisting of hemosiderophages and proliferative capillary blood vessels. The histopathological findings indicated the characteristics of xanthoma.

### LESSONS

Bilateral frontal intracranial xanthoma with associated type II hyperlipidemia is an unusual finding. Despite its rarity and wide variety of radiological presentations, it should be considered one of the differential diagnoses of lesions that develop intracranially and intraaxially. Confirmation with histopathological examination is needed to exclude from other differential diagnoses.

Open access

## Orthostatic hypotension after cervicomedullary junction surgery: illustrative case

### BACKGROUND

Surgery at the cervicomedullary junction carries a risk of damaging vital brainstem functions. Because the nucleus of the solitary tract (NS) is involved in the baroreceptor reflex, damage to its integrity may lead to orthostatic hypotension.

### OBSERVATIONS

A 56-year-old man with a medical history of hypertension, von Hippel-Lindau disease, and previous bilateral adrenalectomy due to pheochromocytoma was referred with symptoms of dysphagia and paralysis of the left vocal cord. Paralysis of the left vagus nerve was suspected. Magnetic resonance imaging revealed a contrast-enhancing cystic process in the cervicomedullary junction. Twenty-three years earlier, the patient had undergone surgical treatment for a hemangioblastoma in the same region. After repeated surgery, the patient temporarily developed orthostatic hypotension. At discharge, the patient no longer needed antihypertensive medication.

### LESSONS

Surgery near the cervicomedullary junction can affect the NS, leading to disruption of the baroreceptor response that regulates blood pressure.

Open access

## Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case

### BACKGROUND

Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma.

### OBSERVATIONS

The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma.

### LESSONS

This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.

Open access

## Primary spinal melanoma: illustrative case

### BACKGROUND

Primary spinal melanoma is extremely rare, accounting for ∼1% of all primary melanomas. Typically presenting insidiously in the thoracic spinal cord, primary spinal melanomas can have an acute presentation due to their propensity to hemorrhage.

### OBSERVATIONS

Despite its rarity, primary spinal melanoma should be included in the differential diagnosis when a hemorrhagic pattern of T1 and T2 intensities is seen on magnetic resonance imaging. Furthermore, the complete diagnosis is crucial because the prognosis of a primary spinal melanoma is considerably more favorable than that of a primary cutaneous melanoma with metastatic spread.

### LESSONS

Resection is the treatment of choice, with some authors advocating for postoperative chemotherapy, immunotherapy, and/or radiation. We describe a case of acute quadriplegia from hemorrhagic primary spinal melanoma requiring resection.

Open access

## Unstable odontoid fractures: technical appraisal of anterior extrapharyangeal open reduction internal fixation for irreducible unstable odontoid fractures. Patient series

### BACKGROUND

Displaced odontoid fractures that are irreducible with traction and have cervicomedullary compression by the displaced distal fracture fragment or deformity caused by facetal malalignment require early realignment and stabilization. Realignment with ultimate solid fracture fusion and atlantoaxial joint fusion, in some situations, are the aims of surgery. Fifteen such patients were treated with direct anterior extrapharyngeal open reduction and realignment of displaced fracture fragments with realignment of the atlantoaxial facets, followed by a variable screw placement (VSP) plate in compression mode across the fracture or anterior atlantoaxial fixation (transarticular screws or atlantoaxial plate screw construct) or both.

### OBSERVATIONS

Anatomical realignment with rigid fixation was achieved in all patients. Fracture fusion without implant failure was observed in 100% of the patients at 6 months, with 1 unrelated mortality. Minimum follow-up has been 6 months in 14 patients and a maximum of 3 years in 4 patients, with 1 unrelated mortality.

### LESSONS

Most irreducible unstable odontoid fractures can be anatomically realigned by anterior extrapharyngeal approach by facet joint manipulation. Plate (VSP) and screws permit rigid fixation in compression mode with 100% fusion. Any associated atlantoaxial instability can be treated from the same exposure.

Restricted access

## The critical importance of a vascularized flap in preventing recurrence after endoscopic repair of spontaneous cerebrospinal fluid leaks and meningoencephaloceles

### OBJECTIVE

Spontaneous CSF leaks into the anterior skull base nasal sinuses are often associated with meningoencephaloceles and occur in patients with idiopathic intracranial hypertension (IIH). Endonasal endoscopic repair has become the primary method of choice for repair. The authors sought to evaluate the success rate of endoscopic closure and to identify predictive factors for CSF leak recurrence.

### METHODS

A consecutive series of endonasally repaired anterior skull base meningoencephaloceles was drawn from a prospectively acquired database. Lumbar punctures were not performed as part of a treatment algorithm. All patients had at least 5 months of follow-up. Chart review and phone calls were used to determine the timing and predictors of recurrence. Demographic information and details of operative technique were correlated with recurrence. Two independent radiologists reviewed all preoperative imaging to identify radiographic markers of IIH, as well as the location and size of the meningoencephalocele.

### RESULTS

From a total of 54 patients there were 5 with recurrences (9.3%), but of the 39 patients in whom a vascularized nasoseptal (n = 31) or turbinate (n = 8) flap was used there were no recurrences (p = 0.0009). The mean time to recurrence was 24.8 months (range 9–38 months). There was a trend to higher BMI in patients whose leak recurred (mean [± SD] 36.6 ± 8.6) compared with those whose leak did not recur (31.8 ± 7.4; p = 0.182). Although the lateral recess of the sphenoid sinus was the most common site of meningoencephalocele, the fovea ethmoidalis was the most common site in recurrent cases (80%; p = 0.013). However, a vascularized flap was used in significantly more patients with sphenoid (78.3%) defects than in patients with fovea ethmoidalis (28.6%) defects (Fisher’s exact test, p = 0.005). Radiographic signs of IIH were equally present in all patients whose leak recurred (75%) compared with patients whose leak did not recur (63.3%); however, an enlarged Meckel cave was present in 100% (2/2) of patients whose leaks recurred compared with 13.3% (4/30) of patients whose leaks did not recur (p = 0.03). The average meningoencephalocele diameter tended to be larger (1.73 ± 1.3 cm) in patients with recurrence compared to those without recurrence (1.2 ± 0.66 cm; p = 0.22). A ventriculoperitoneal shunt was already in place in 3 patients, placed perioperatively in 5, and placed at recurrence in 2, none of whose leaks recurred.

### CONCLUSIONS

Recurrence after endonasal repair of spontaneous CSF leaks from meningoencephaloceles can be dramatically reduced with the use of a vascularized flap. Although failures of endonasal repair tend to occur in patients who have higher BMI, larger brain herniations, and no CSF diversion, the lack of vascularized flap was the single most important risk factor predictive of failure.

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## A critical reappraisal of corticospinal tract somatotopy and its role in traumatic cervical spinal cord syndromes

The corticospinal tract (CST) is the preeminent voluntary motor pathway that controls human movements. Consequently, long-standing interest has focused on CST location and function in order to understand both loss and recovery of neurological function after incomplete cervical spinal cord injury, such as traumatic central cord syndrome. The hallmark clinical finding is paresis of the hands and upper-extremity function with retention of lower-extremity movements, which has been attributed to injury and the sparing of specific CST fibers. In contrast to historical concepts that proposed somatotopic (laminar) CST organization, the current narrative summarizes the accumulated evidence that 1) there is no somatotopic organization of the corticospinal tract within the spinal cord in humans and 2) the CST is critically important for hand function. The evidence includes data from 1) tract-tracing studies of the central nervous system and in vivo MRI studies of both humans and nonhuman primates, 2) selective ablative studies of the CST in primates, 3) evolutionary assessments of the CST in mammals, and 4) neuropathological examinations of patients after incomplete cervical spinal cord injury involving the CST and prominent arm and hand dysfunction. Acute traumatic central cord syndrome is characterized by prominent upper-extremity dysfunction, which has been falsely predicated on pinpoint injury to an assumed CST layer that specifically innervates the hand muscles. Given the evidence surveyed herein, the pathophysiological mechanism is most likely related to diffuse injury to the CST that plays a critically important role in hand function.

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## Disparities in access to surgery for glioblastoma multiforme at high-volume Commission on Cancer–accredited hospitals in the United States

### OBJECTIVE

Although it has been shown that surgery for glioblastoma (GBM) at high-volume facilities (HVFs) may be associated with better postoperative outcomes, the use of such hospitals may not be equally distributed. The authors aimed to evaluate racial and socioeconomic differences in access to surgery for GBM at high-volume Commission on Cancer (CoC)–accredited hospitals.

### METHODS

The National Cancer Database was queried for patients with GBM that was newly diagnosed between 2004 and 2015. Patients who received no surgical intervention or those who received surgical intervention at a site other than the reporting facility were excluded. Annual surgical case volume was calculated for each hospital, with volume ≥ 90th percentile defined as an HVF. Multivariable logistic regression was performed to identify patient-level predictors for undergoing surgery at an HVF. Furthermore, multiple subgroup analyses were performed to determine the adjusted odds ratio of the likelihood of undergoing surgery at an HVF in 2016 as compared to 2004 for each patient subpopulation (by age, race, sex, educational group, etc.).

### RESULTS

A total of 51,859 patients were included, with 10.7% (n = 5562) undergoing surgery at an HVF. On multivariable analysis, Hispanic White patients (OR 0.58, 95% CI 0.49–0.69, p < 0.001) were found to have significantly lower odds of undergoing surgery at an HVF (reference = non-Hispanic White). In addition, patients from a rural residential location (OR 0.55, 95% CI 0.41–0.72, p < 0.001; reference = metropolitan); patients with nonprivate insurance status (Medicare [OR 0.78, 95% CI 0.71–0.86, p < 0.001], Medicaid [OR 0.68, 95% CI 0.60–0.78, p < 0001], other government insurance [OR 0.68, 95% CI 0.52–0.86, p = 0.002], or who were uninsured [OR 0.61, 95% CI 0.51–0.72, p < 0.001]); and lower-income patients ($50,354–$63,332 [OR 0.68, 95% CI 0.63–0.74, p < 0.001], $40,227–$50,353 [OR 0.84, 95% CI 0.76–0.92, p < 0.001]; reference = ≥ \$63,333) were also found to be significantly associated with a lower likelihood of surgery at an HVF. Subgroup analyses revealed that elderly patients (age ≥ 65 years), both male and female patients and non-Hispanic White patients, and those with private insurance, Medicare, metropolitan residential location, median zip code–level household income in the first and second quartiles, and educational attainment in the first and third quartiles had increased odds of undergoing surgery at an HVF in 2016 compared to 2004 (all p ≤ 0.05). On the other hand, patients with other governmental insurance, patients with a rural residence, and those from a non-White racial category did not show a significant difference in odds of surgery at an HVF over time (all p > 0.05).

### CONCLUSIONS

The present analysis from the National Cancer Database revealed significant disparities in access to surgery at an HVF for GBM within the United States. Furthermore, there was evidence that these racial and socioeconomic disparities may have widened between 2004 and 2016. The findings should assist health policy makers in the development of strategies for improving access to HVFs for racially and socioeconomically disadvantaged populations.

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## Endoscopic decompression for the treatment of lumbar spinal stenosis: an updated systematic review and meta-analysis

### OBJECTIVE

Lumbar stenosis treatment has evolved with the introduction of minimally invasive surgery (MIS) techniques. Endoscopic methods take the concepts applied to MIS a step further, with multiple studies showing that endoscopic techniques have outcomes that are similar to those of more traditional approaches. The aim of this study was to perform an updated meta-analysis and systematic review of studies comparing the outcomes between endoscopic (uni- and biportal) and microscopic techniques for the treatment of lumbar stenosis.

### METHODS

Following PRISMA guidelines, a systematic search was performed using the Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Ovid Embase, and PubMed databases from their dates of inception to December 14, 2020. All identified articles were then systematically screened against the following inclusion criteria: 1) studies comparing endoscopic (either uniportal or biportal) with minimally invasive approaches, 2) patient age ≥ 18 years, and 3) diagnosis of lumbar spinal stenosis. Bias was assessed using quality assessment criteria and funnel plots. Meta-analysis using a random-effects model was used to synthesize the metadata.

### RESULTS

From a total of 470 studies, 14 underwent full-text assessment. Of these 14 studies, 13 comparative studies were included for quantitative analysis, totaling 1406 procedures satisfying all criteria for selection. Regarding postoperative back pain, 9 studies showed that endoscopic methods resulted in significantly lower pain scores compared with MIS (mean difference [MD] −1.0, 95% CI −1.6 to −0.4, p < 0.01). The length of stay data were reported by 7 studies, with endoscopic methods associated with a significantly shorter length of stay versus the MIS technique (MD −2.1 days, 95% CI −2.7 to −1.4, p < 0.01). There was no significant difference with respect to leg visual analog scale scores, Oswestry Disability Index scores, blood loss, surgical time, and complications, and there were not any significant quality or bias concerns.

### CONCLUSIONS

Both endoscopic and MIS techniques are safe and effective methods for treating patients with symptomatic lumbar stenosis. Patients who undergo endoscopic surgery seem to report less postoperative low-back pain and significantly reduced hospital stay with a trend toward less perioperative blood loss. Future large prospective randomized trials are needed to confirm the findings in this study.

Restricted access

## Extended endoscopic transorbital approach with superior-lateral orbital rim osteotomy: cadaveric feasibility study and clinical implications (SevEN-007)

### OBJECTIVE

The endoscopic transorbital approach (ETOA) has been developed, permitting a new surgical corridor. Due to the vertical limitation of the ETOA, some lesions of the anterior cranial fossa are difficult to access. The ETOA with superior-lateral orbital rim (SLOR) osteotomy can achieve surgical freedom of vertical as well as horizontal movement. The purpose of this study was to confirm the feasibility of the ETOA with SLOR osteotomy.

### METHODS

Anatomical dissections were performed in 5 cadaveric heads with a neuroendoscope and neuronavigation system. ETOA with SLOR osteotomy was performed on one side of the head, and ETOA with lateral orbital rim (LOR) osteotomy was performed on the other side. After analysis of the results of the cadaveric study, the ETOA with SLOR osteotomy was applied in 6 clinical cases.

### RESULTS

The horizontal and vertical movement range through ETOA with SLOR osteotomy (43.8° ± 7.49° and 36.1° ± 3.32°, respectively) was improved over ETOA with LOR osteotomy (31.8° ± 5.49° and 23.3° ± 1.34°, respectively) (p < 0.01). Surgical freedom through ETOA with SLOR osteotomy (6025.1 ± 220.1 mm3) was increased relative to ETOA with LOR osteotomy (4191.3 ± 57.2 mm3) (p < 0.01); these values are expressed as the mean ± SD. Access levels of ETOA with SLOR osteotomy were comfortable, including anterior skull base lesion and superior orbital area. The view range of the endoscope for anterior skull base lesions was increased through ETOA with SLOR osteotomy. After SLOR osteotomy, the space for moving surgical instruments and the endoscope was widened. Anterior clinoidectomy could be achieved successfully using ETOA with SLOR osteotomy.

The authors performed ETOA with SLOR osteotomy in 6 cases of brain tumor. In all 6 cases, complete removal of the tumor was successfully accomplished. In the 3 cases of anterior clinoidal meningioma, anterior clinoidectomy was performed easily and safely, and manipulation of the extended dural margin and origin dura mater was possible. There was no complication related to this approach.

### CONCLUSIONS

The authors evaluated the clinical feasibility of ETOA with SLOR osteotomy based on a cadaveric study. ETOA with SLOR osteotomy could be applied to more diverse disease groups that do not permit conventional ETOA or to cases in which surgical application is challenging. ETOA with SLOR osteotomy might serve as an opportunity to broaden the indication for the ETOA.