Intracranial extension of temporomandibular joint (TMJ) ganglion cysts is very rare. Two previously reported cases presented clinically due to effects on cranial nerves and had obvious association with the TMJ on imaging. To the authors’ knowledge, intracranial extension of a TMJ ganglion cyst presenting with seizures and mimicking a primary brain tumor has not been previously reported. The patient underwent resection of a presumptive primary cystic temporal lobe tumor, but the lesion had histopathological features of a nonneoplastic cyst with a myxoid content. He was followed with serial imaging for 5 years before regrowth of the lesion caused new episodes of seizures requiring a repeat operation, during which the transdural defect was repaired after the adjacent segment of the TMJ was curetted. A thorough review of all imaging studies and the histopathological findings from the repeat operation led to the correct diagnosis of a TMJ ganglion cyst. This case highlights an unusual presentation of this rare lesion, as well as its potential for recurrence. TMJ ganglion cysts should be included in the differential diagnosis of cystic tumors involving the anterior temporal lobe, presenting with or without seizures. Focused imaging evaluation of the TMJ can be helpful to rule out the possible role of associated TMJ lesions.
Aaron P. Kamer, Jose M. Bonnin, Robert J. Spinner and Aaron A. Cohen-Gadol
Clint M. Alfaro, Valentina Pirro, Michael F. Keating, Eyas M. Hattab, R. Graham Cooks and Aaron A. Cohen-Gadol
The authors describe a rapid intraoperative ambient ionization mass spectrometry (MS) method for determining isocitrate dehydrogenase (IDH) mutation status from glioma tissue biopsies. This method offers new glioma management options and may impact extent of resection goals. Assessment of the IDH mutation is key for accurate glioma diagnosis, particularly for differentiating diffuse glioma from other neoplastic and reactive inflammatory conditions, a challenge for the standard intraoperative diagnostic consultation that relies solely on morphology.
Banked glioma specimens (n = 37) were analyzed by desorption electrospray ionization–MS (DESI-MS) to develop a diagnostic method to detect the known altered oncometabolite in IDH-mutant gliomas, 2-hydroxyglutarate (2HG). The method was used intraoperatively to analyze tissue smears obtained from glioma patients undergoing resection and to rapidly diagnose IDH mutation status (< 5 minutes). Fifty-one tumor core biopsies from 25 patients (14 wild type [WT] and 11 mutant) were examined and data were analyzed using analysis of variance and receiver operating characteristic curve analysis.
The optimized DESI-MS method discriminated between IDH-WT and IDH-mutant gliomas, with an average sensitivity and specificity of 100%. The average normalized DESI-MS 2HG signal was an order of magnitude higher in IDH-mutant glioma than in IDH-WT glioma. The DESI 2HG signal intensities correlated with independently measured 2HG concentrations (R2 = 0.98). In 1 case, an IDH1 R132H–mutant glioma was misdiagnosed as a demyelinating condition by frozen section histology during the intraoperative consultation, and no resection was performed pending the final pathology report. A second craniotomy and tumor resection was performed after the final pathology provided a diagnosis most consistent with an IDH-mutant glioblastoma. During the second craniotomy, high levels of 2HG in the tumor core biopsies were detected.
This study demonstrates the capability to differentiate rapidly between IDH-mutant gliomas and IDH-WT conditions by DESI-MS during tumor resection. DESI-MS analysis of tissue smears is simple and can be easily integrated into the standard intraoperative pathology consultation. This approach may aid in solving differential diagnosis problems associated with low-grade gliomas and could influence intraoperative decisions regarding extent of resection, ultimately improving patient outcome. Research is ongoing to expand the patient cohort, systematically validate the DESI-MS method, and investigate the relationships between 2HG and tumor heterogeneity.
Aaron A. Cohen-Gadol
Lorenzo Rinaldo, David S. Priemer, Alexander O. Vortmeyer, Aaron A. Cohen-Gadol, Daniel J. Brat, Anita Mahajan, Caterina Giannini and Terry C. Burns
Chordomas are neoplasms that typically arise from midline skeletal structures and rarely originate within the intradural compartment of the CNS. A chordoma arising from the corpus callosum has not been previously described. The authors report the surgical management of a chordoma originating within the splenium of the corpus callosum. To determine the incidence and distribution of intracranial intradural chordoma, a literature search for additional cases was performed. MEDLINE was searched using the MeSH keyword “chordoma,” yielding 2010 articles. These articles were screened for cases of primary intradural chordoma rostral to the craniocervical junction, which led to the identification of 46 relevant articles. The authors report the case of a 69-year-old man who initially presented with nonspecific neurological symptoms including spatial disorientation and cognitive decline. These symptoms eventually prompted intracranial imaging, including MRI, which revealed a ring-enhancing, heterogeneous, cystic mass localized within the splenium of the corpus callosum and extending into the bilateral ventricles. The lesion was believed to represent a high-grade glioma and the patient underwent a left interhemispheric approach and subtotal resection. After pathologic evaluation confirmed a diagnosis of an anaplastic chordoma, the patient underwent further resection. A gross-total resection (GTR) was achieved with a transfalcine approach to the contralateral portion of the tumor. Postoperatively, the patient had a partial left homonymous quadrantanopsia, but was otherwise at his neurological baseline. Proton beam radiotherapy was performed to the resection cavity but diffuse intraventricular disease ensued. The results of a literature search suggest that a chordoma arising in the corpus callosum has not been previously described. The present case demonstrates that chordomas can occur in the corpus callosum, and illustrates the utility of a transfalcine approach for GTR of lesions in this location, as well as the need for improved strategies to prevent intraventricular dissemination.
Frank J. Attenello, Ian A. Buchanan, Timothy Wen, Daniel A. Donoho, Shirley McCartney, Steven Y. Cen, Alexander A. Khalessi, Aaron A. Cohen-Gadol, Joseph S. Cheng, William J. Mack, Clemens M. Schirmer, Karin R. Swartz, J. Adair Prall, Ann R. Stroink, Steven L. Giannotta and Paul Klimo Jr.
Excessive dissatisfaction and stress among physicians can precipitate burnout, which results in diminished productivity, quality of care, and patient satisfaction and treatment adherence. Given the multiplicity of its harms and detriments to workforce retention and in light of the growing physician shortage, burnout has garnered much attention in recent years. Using a national survey, the authors formally evaluated burnout among neurosurgery trainees.
An 86-item questionnaire was disseminated to residents in the American Association of Neurological Surgeons database between June and November 2015. Questions evaluated personal and workplace stressors, mentorship, career satisfaction, and burnout. Burnout was assessed using the previously validated Maslach Burnout Inventory. Factors associated with burnout were determined using univariate and multivariate logistic regression.
The response rate with completed surveys was 21% (346/1643). The majority of residents were male (78%), 26–35 years old (92%), in a stable relationship (70%), and without children (73%). Respondents were equally distributed across all residency years. Eighty-one percent of residents were satisfied with their career choice, although 41% had at some point given serious thought to quitting. The overall burnout rate was 67%. In the multivariate analysis, notable factors associated with burnout included inadequate operating room exposure (OR 7.57, p = 0.011), hostile faculty (OR 4.07, p = 0.008), and social stressors outside of work (OR 4.52, p = 0.008). Meaningful mentorship was protective against burnout in the multivariate regression models (OR 0.338, p = 0.031).
Rates of burnout and career satisfaction are paradoxically high among neurosurgery trainees. While several factors were predictive of burnout, including inadequate operative exposure and social stressors, meaningful mentorship proved to be protective against burnout. The documented negative effects of burnout on patient care and health care economics necessitate further studies for potential solutions to curb its rise.
Ryan P. Morton, Louis J. Kim and Laligam N. Sekhar
Mahdi Malekpour, Charles Kulwin, Bradley N. Bohnstedt, Golnar Radmand, Rishabh Sethia, Stephen K. Mendenhall, Jonathan Weyhenmeyer, Benjamin K. Hendricks, Thomas Leipzig, Troy D. Payner, Mitesh V. Shah, John Scott, Andrew DeNardo, Daniel Sahlein and Aaron A. Cohen-Gadol
Aneurysmal rebleeding before definitive obliteration of the aneurysm is a cause of mortality and morbidity. There are limited data on the role of short-term antifibrinolytic therapy among patients undergoing endovascular intervention.
All consecutive patients receiving endovascular therapy for their ruptured saccular aneurysm at the authors' institution between 2000 and 2011 were included in this study. These patients underwent endovascular coiling of their aneurysm within 72 hours of admission. In patients receiving ε-aminocaproic acid (EACA), the EACA administration was continued until the time of the endovascular procedure. Complications and clinical outcomes of endovascular treatment after aneurysmal subarachnoid hemorrhage (aSAH) were compared between EACA-treated and untreated patients.
During the 12-year study period, 341 patients underwent endovascular coiling. Short-term EACA treatment was administered in 146 patients and was withheld in the other 195 patients. EACA treatment did not change the risk of preinterventional rebleeding in this study (OR 0.782, 95% CI 0.176–3.480; p = 0.747). Moreover, EACA treatment did not increase the rate of thromboembolic events. On the other hand, patients who received EACA treatment had a significantly longer duration of hospital stay compared with their counterparts who were not treated with EACA (median 19 days, interquartile range [IQR] 12.5–30 days vs median 14 days, IQR 10–23 days; p < 0.001). EACA treatment was associated with increased odds of shunt requirement (OR 2.047, 95% CI 1.043–4.018; p = 0.037) and decreased odds of developing cardiac complications (OR 0.138, 95% CI 0.031–0.604; p = 0.009) and respiratory insufficiency (OR 0.471, 95% CI 0.239–0.926; p = 0.029). Short-term EACA treatment did not affect the Glasgow Outcome Scale score at discharge, 6 months, or 1 year following discharge.
In this study, short-term EACA treatment in patients who suffered from aSAH and received endovascular aneurysm repair did not decrease the risk of preinterventional rebleeding or increase the risk of thrombotic events. EACA did not affect outcome. Randomized clinical trials are required to provide robust clinical recommendation on short-term use of EACA.
René Post, Bert A. Coert, W. Peter Vandertop, Dagmar Verbaan and Menno R. Germans
Mason A. Brown, Jonathan Parish, Cristian F. Guandique, Troy D. Payner, Terry Horner, Thomas Leipzig, Karishma V. Rupani, Richard Kim, Bradley N. Bohnstedt and Aaron A. Cohen-Gadol
With the recent evolution of endovascular therapies, objective evaluation of the efficacy of clip ligation for cerebral aneurysms should be performed. This study was undertaken to evaluate the durability of microsurgical clip ligation, identify risk factors for recurrence, and assess the need for long-term follow-up imaging.
A retrospective review of medical records identified 616 consecutive patients (156 male and 460 female patients; mean age 48.4 ± 12.4 years; range 6–90 years) who underwent microsurgical clip ligation and follow-up imaging at least 1 year after discharge between 1990 and 2010 at our institution. Of a total of 926 aneurysms in 616 patients, 758 aneurysms were microsurgically clip-ligated. At presentation, 431 of these aneurysms were ruptured and 327 aneurysms were unruptured. All patients underwent postoperative baseline imaging within the 1st month of their operation. A logistic regression analysis was performed to identify which variables are more likely to predict recurrence.
Late follow-up angiographic imaging was obtained at a mean of 7.2 ± 4.7 years postdischarge (median 5.7 years; range 1–23 years). Of the 699 clipped aneurysms without residua, late follow-up angiography revealed only 1 (0.14%) recurrent aneurysm. Of the 59 residual aneurysms that remained after initial clip ligation on early postoperative imaging, 8 (13.6%) demonstrated growth. All of these aneurysms required treatment. None of the recurrences were due to broken or delayed displacement of clips. A total of 111 patients presented with multiple aneurysms. De novo aneurysm formation occurred in 8 (0.97%) patients, all of whom initially presented with multiple aneurysms.
This study provides additional evidence to support the long-term efficacy of aneurysm clip ligation. The chance of aneurysm recurrence after complete clip ligation is very small. However, there is a regrowth risk of 1.83% per year for aneurysm remnants after incomplete clip ligation. These findings support the necessity for continued followup, late angiographic imaging, and the potential need for further intervention of incompletely ligated aneurysms. Furthermore, completely clip-ligated aneurysms may not require additional surveillance imaging unless multiple aneurysms were evident at presentation.