Link, Michael J.

Abstract Introduction Neurenteric cysts (NECs) are rare, congenital lesions lined by endodermal cell-derived columnar or cuboidal epithelium. Based on previous studies, gross total removal of the capsule has been presumed to be the ideal surgical goal. Objective This series was undertaken to further understand the risk of recurrence based on the extent of capsule resection. Methods Records were retrospectively reviewed for all patients with radiographic or pathological evidence of intracranial NEC from 1996 to 2021. Results A total of eight patients were identified; four of eight (50%) presented with headache, and four had signs of one or more cranial nerve syndromes. One patient (13%) presented with third nerve palsy, one (13%) had sixth nerve palsy, and two (25%) with hemifacial spasm. One patient (13%) presented with signs of obstructive hydrocephalus. Magnetic resonance imaging demonstrated T2 hyper- or isointense lesions. Diffusion-weighted imaging was negative in all patients (100%) and T1 contrast-enhanced imaging demonstrated minimal rim enhancement in two patients (25%). In three of eight (38%), a gross total resection (GTR) was achieved, while in four (50%), a near-total resection, and in one (13%), a decompression was performed. Recurrences occurred in two (25%) patients, one with decompression and another with near-total resection, among these 1/2 required repeat surgery after a mean follow-up of 77 months. Conclusion In this series, none from GTR group demonstrated recurrence, while 40% of those receiving less than GTR recurred, underpinning the importance of maximally safe resection in these patients. Overall patients did well without major morbidity from surgery.

OBJECTIVE The authors’ objective was to reevaluate the role of microsurgery for epidermoid tumors by examining the associations between extent of resection (EOR), tumor control, and clinical outcomes. METHODS This was a retrospective study of patients with microsurgically treated intracranial epidermoid tumors. The recurrence-free and intervention-free rates were calculated using the Kaplan-Meier method. EOR was graded as gross-total resection (GTR) (total resection without residual on MRI), near-total resection (NTR) (a cyst lining was left in place), subtotal resection (STR) (> 90% resection), and partial resection (PR) (any other suboptimal resection) and used to stratify outcomes. RESULTS Sixty-three patients with mean clinical and radiological follow-up periods of 87.3 and 81.8 months, respectively, were included. Sixteen patients underwent second resections, and 5 underwent third resections. The rates of GTR/NTR, STR, and PR were 43%, 35%, and 22%, respectively, for the initial resections; 44%, 13%, and 44% for the second resections; and 40%, 0%, and 60% for the third resections (p < 0.001). The 5- and 10-year cumulative recurrence-free rates after initial resection were 64% and 32%, respectively. When stratified according to EOR, the 10-year recurrence-free rate after GTR/NTR was marginally better than that after STR (61% vs 35%, p = 0.130) and significantly better than that after PR (61% vs 0%, p < 0.001). The recurrence-free rates after initial microsurgery were marginally better than those after second surgery (p = 0.102) and third surgery (p = 0.065). The 5- and 10-year cumulative intervention-free rates after initial resection were 91% and 58%, respectively. When stratified according to EOR, the 10-year intervention-free rate after GTR/NTR was significantly better than that after STR (100% vs 51%, p = 0.022) and PR (100% vs 27%, p < 0.001). The 5-year intervention-free rate after initial surgery was marginally better than that after second surgery (52%, p = 0.088) and significantly better than that after third surgery (0%, p = 0.004). After initial, second, and third resections, permanent neurological complications were observed in 6 (10%), 1 (6%), and 1 (20%) patients, respectively. At the last follow-up visit, 82%, 23%, and 7% of patients were free from radiological recurrence after GTR/NTR, STR, and PR as the initial surgical procedure, respectively. CONCLUSIONS GTR/NTR seems to contribute to better disease control without significantly impairing functional status. Initial resection offers the best chance to achieve better EOR, leading to better disease control.

Abstract Epidermoid cysts are rare, benign lesions that result from inclusion of ectodermal elements during neural tube closure. 1 Cysts are composed of desquamated epithelial cells and restrict diffusion on magnetic resonance imaging (MRI). 2,3 Symptoms are attributable to anatomic location. 4,5 In this video, we illustrate the surgical treatment of an epidermoid cyst located in the right cerebellopontine angle, petrous apex, and Meckel's cave. The patient, a 33-yr-old female with right-sided V1 trigeminal hypoesthesia, underwent surveillance imaging for 2 yr. However, she developed progressive V1 and V2 trigeminal hypoesthesia and imaging revealed enlargement of the lesion. Therefore, surgical resection was pursued. The patient consented to the procedure. The patient underwent a right middle fossa craniotomy and anterior petrosectomy. After identifying the greater superficial petrosal nerve and cutting the middle meningeal artery as it exited foramen spinosum, Kawase's triangle was drilled, and the dura over Meckel's cave and the subtemporal dura were opened. The lesion was resected, taking care to preserve the trigeminal nerve and the basilar artery. A retrosigmoid craniotomy was then fashioned. The cyst and its capsule were dissected off the brainstem and cranial nerves utilizing natural corridors between the trigeminal and vestibulocochlear nerves as well as between the facial and lower cranial nerves. Gross total resection was confirmed on postoperative MRI, and she was discharged home on postoperative day 5. Three months after surgery, she underwent formal pinprick testing, which revealed 95% loss of sensation in V1, 20% loss in V2, and normal sensation in V3. Three-month postoperative MRI showed no residual tumor.

OBJECTIVE There is a reported elevated risk of cerebral aneurysms in patients with intracranial dural arteriovenous fistulas (dAVFs). However, the natural history, rate of spontaneous regression, and ideal treatment regimen are not well characterized. In this study, the authors aimed to describe the characteristics of patients with dAVFs and intracranial aneurysms and propose a classification system. METHODS The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database from 12 centers was retrospectively reviewed. Analysis was performed to compare dAVF patients with (dAVF+ cohort) and without (dAVF-only cohort) concomitant aneurysm. Aneurysms were categorized based on location as a dAVF flow-related aneurysm (FRA) or a dAVF non–flow-related aneurysm (NFRA), with further classification as extra- or intradural. Patients with traumatic pseudoaneurysms or aneurysms with associated arteriovenous malformations were excluded from the analysis. Patient demographics, dAVF anatomical information, aneurysm information, and follow-up data were collected. RESULTS Of the 1077 patients, 1043 were eligible for inclusion, comprising 978 (93.8%) and 65 (6.2%) in the dAVF-only and dAVF+ cohorts, respectively. There were 96 aneurysms in the dAVF+ cohort; 10 patients (1%) harbored 12 FRAs, and 55 patients (5.3%) harbored 84 NFRAs. Dural AVF+ patients had higher rates of smoking (59.3% vs 35.2%, p < 0.001) and illicit drug use (5.8% vs 1.5%, p = 0.02). Sixteen dAVF+ patients (24.6%) presented with aneurysm rupture, which represented 16.7% of the total aneurysms. One patient (1.5%) had aneurysm rupture during follow-up. Patients with dAVF+ were more likely to have a dAVF located in nonconventional locations, less likely to have arterial supply to the dAVF from external carotid artery branches, and more likely to have supply from pial branches. Rates of cortical venous drainage and Borden type distributions were comparable between cohorts. A minority (12.5%) of aneurysms were FRAs. The majority of the aneurysms underwent treatment via either endovascular (36.5%) or microsurgical (15.6%) technique. A small proportion of aneurysms managed conservatively either with or without dAVF treatment spontaneously regressed (6.2%). CONCLUSIONS Patients with dAVF have a similar risk of harboring a concomitant intracranial aneurysm unrelated to the dAVF (5.3%) compared with the general population (approximately 2%–5%) and a rare risk (0.9%) of harboring an FRA. Only 50% of FRAs are intradural. Dural AVF+ patients have differences in dAVF angioarchitecture. A subset of dAVF+ patients harbor FRAs that may regress after dAVF treatment.

Background Jugular paragangliomas represent a surgical challenge due to their vascularity and proximity to vital neurovascular structures. Preoperative embolization aids in reducing intraoperative blood loss, transfusion requirements, and improves surgical visualization. Several embolization agents have been used. Objective The aim of this study is to evaluate the safety and efficacy of PVA in pre-operative embolization of jugular paragangliomas. Methods A retrospective review of all patients who underwent jugular paraganglioma resection with pre-operative embolization between 2000 and 2020 was performed. Pre-operative data including baseline patient and tumor characteristics were documented. Outcomes of preoperative embolization including extent of devascularization and post-embolization complications were recorded. Early and long-term postoperative outcomes were reported. Results Twenty-nine patients met study criteria with a median age of 38 years. Average tumor size was 3.4±1.8 cm. The most commonly encountered arterial feeder was the ascending pharyngeal artery followed by the posterior auricular artery. More than 50% reduction in tumor blush was achieved in 25 patients (86.2%). None of the patients experienced new or worsening cranial neuropathy following embolization. Gross total or Near total resection was achieved in 13 patients (44.8%). A STR or NTR was chosen in these patients to preserve cranial nerve function or large vessel integrity. Average intraoperative estimated blood loss was 888 ml, 9 patients (31%) required intra-operative transfusion of blood products. Extent of resection and post-operative complications did not correlate with extent of devascularization. Conclusion Pre-operative embolization of jugular paraganglioma tumors with PVA particles is an effective strategy with a high safety profile.