Van Gompel, Jamie J

Abstract BACKGROUND The Food and Drug Administration approved the deep brain stimulation of the anterior nucleus of the thalamus (ANT-DBS) as an adjunctive therapy for drug-resistant epilepsy (DRE) in the United States in 2018. The DBS Therapy for Epilepsy Post-Approval Study is further evaluating the safety and effectiveness of ANT-DBS among different patients’ groups. For this study, devices for vagus nerve stimulation (VNS) must be removed prior to enrolment. OBJECTIVE To investigate the outcomes of concomitant ANT-DBS and VNS treatment for DRE. METHODS A retrospective analysis was performed for 33 patients who underwent ANT-DBS using previous VNS to define distinct subgroups: standard ANT-DBS (9 subjects), ANT-DBS with functional VNS (12 subjects), and ANT-DBS with the VNS implantable pulse generator explanted or turned off at the time of the DBS (12 subjects). Effectiveness and safety data were analyzed across the whole population and among subgroups. RESULTS A mean decrease in seizure frequency of 55% was observed after a mean follow-up of 25.5 mo. Approximately 67% of patients experienced ≥50% reduction in seizure frequency. Seizure reduction percentage was not significantly different among groups. Approximately 50% of subjects with no appreciable improvement and 75% of those who showed benefit after VNS (including improvement in seizure frequency, seizure severity, and seizure duration or quality of life) achieved a seizure reduction ≥50% after ANT-DBS surgery. There were no complications related to concomitant VNS and ANT-DBS. CONCLUSION ANT-DBS for DRE provides excellent results despite previous and ongoing VNS therapy. Removal of VNS does not appear to be necessary before ANT-DBS.

Abstract Objectives Juvenile nasopharyngeal angiofibromas (JNAs) are uncommon tumors with an evolving treatment paradigm. The objective of this study was to compare our prior experience reported in 2005 with our most contemporary series to compare practice improvements and the impact of expanded endonasal procedures. Design Retrospective review comparing a contemporary 22 patients with JNA who underwent surgical management between 2005 and 2019, compared with a historical cohort of 65 patients from the same center. Results The most common presenting symptom was epistaxis (68%). The median maximum tumor diameter was 4.4 cm. All patients underwent preoperative embolization. An endoscopic endonasal approach (EEA) was used in 18 patients (82%), compared with 9% in the series prior to 2005. Gross total resection was achieved in all patients. The median estimated blood loss was 175 and 350 mL for EEA and open (transfacial) cases, respectively. Only two patients (9%) required a blood transfusion compared with 52% on the previous series. The median follow-up was 19 months. The overall recurrence rate was 9% in this series and 24% in the previous series. No patient required radiation therapy in follow-up compared with 3% in our historical cohort. Conclusion There have been significant changes regarding the management of patients with JNA compared with the previous Mayo Clinic experience. The EEA has become the preferred route over the transfacial approaches to treat JNA in selected patients who do not have intracranial extension. Preoperative embolization has aided in reducing the postoperative transfusion rates.

Objective/HypothesisAneurysmal bone cysts (ABCs) are benign, lytic bone lesions, which rarely present in the paranasal sinuses. There is no published consensus on the appropriate diagnostic or treatment approach. Our objective was to elucidate the clinical behavior, treatment, and outcomes for patients with ABCs of the paranasal sinuses (psABCs).Study DesignRetrospective case series and system review of the literature.MethodsA retrospective chart review was performed to identify patients evaluated at the authors' institution with psABC and consolidated with literature reports of psABC.ResultsEighty‐nine patients met inclusion criteria. The most common presenting symptom was painless facial swelling (n = 35, 39%). The ethmoid sinuses were the most common site involved (n = 55, 62%). Surgical approach was reported in 74 cases including 20 endoscopic and 54 with an open or combined approach. Fifty‐nine patients (71%) underwent gross total resection. Follow up data was reported for 67 patients. Fifteen patients (22%) were found to have recurrence or progression at follow up; 10/15 (66%) patients within the first 12 months, 14/15 (93%) within the first 24 months, and 1/15 (7%) greater than 24 months after treatment. Patients were more likely to have recurrence/progression if they presented with nasal discharge (P = .05), proptosis (P = .01), or orbital involvement (P = .03).ConclusionspsABCs typically present with painless swelling or nasal obstruction. Orbital involvement is a negative prognostic indicator with these patients more likely to have recurrence after treatment. Recurrence or progression of disease is most likely to occur within 2 years after treatment. Therefore, patients should be monitored closely during this time.Level of Evidence4 Laryngoscope, 131:E2525–E2533, 2021

Abstract Dr Thoralf Sundt III joined the department of neurosurgery at Mayo Clinic in 1969 and served as chairman from 1980 until his death in 1992. Dr Sundt was a pioneer in the field of cerebrovascular neurosurgery and among his many contributions to neurosurgical technology was the introduction of the Sundt clip graft (Codman, Raynham, Massachusetts), which was developed in partnership with Dr George Kees. This device is a vessel-encircling Teflon or Dacron graft loaded on a circular metallic spring initially designed to repair injuries to vessel walls during intracranial aneurysm surgery, 1 which was not an uncommon complication resulting from the use of contemporary aneurysm clips. 2 When used for this purpose, the clip graft has demonstrated both safety and efficacy in a modern series of surgical treatment of intracranial aneurysm. 3 An additional application of the clip graft is the direct clipping of aneurysms, particularly side-wall aneurysms, though Dr Sundt recognized that the potential to occlude perforating arteries adjacent to the aneurysm neck would limit the general use of the clip graft for aneurysm clipping. 2 In the following video, we present archival footage of Dr Sundt utilizing the Sundt clip graft during intracranial aneurysm surgery. The 3 cases are of the treatment of posterior communicating, middle cerebral, and anterior communicating artery aneurysms and depict the potential applications of the clip graft during aneurysm surgery. We also present footage of animal experiments Dr Sundt performed to validate the use of the clip graft. This footage likely represents some of the first microscopic neurosurgical recordings ever made. The patients consented to the procedure and to presentation of video recordings at the time of initial surgery. Image of Sundt at 0:11, Reproduced with permission from the American Association of Neurological Surgeons, 5550 Meadowbrook Dr, Rolling Meadows, IL 60008. Figures at 2:26 and 2:28, from Park PJ, Meyer FB, The Sundt clip graft, Neurosurgery , 2010;66(6 suppl operative):300-305, by permission of the Congress of Neurological Surgeons. Figure at 2:35, ©2009 Mayo, Clingman. Used with permission.

Abstract Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular benign tumor that originates in the sphenopalatine foramen and often spreads to adjacent compartments. 1 Microsurgical resection with preoperative embolization remains the treatment of choice. 2 We present a case of a large JNA involving multiple compartments. The patient is a 20-yr-old male who presented with long-term right nasal congestion. The MRI demonstrated a large enhancing mass that extended from the right nasal cavity and nasopharynx into the right pterygopalatine fossa (PPF), infratemporal fossa (ITF), and parapharyngeal space. Preoperative angiogram for embolization showed a highly vascular tumor with blood supply mainly from the internal maxillary artery and about 10% from a persistent mandibular branch of the internal carotid artery. Based on the UPMC JNA staging system, this tumor was a stage IV. 2 A combined anterior transmaxillary (Caldwell-Luc) with an endoscopic endonasal transpterygoid approach was performed. The addition of the anterior transmaxillary approach increases the surgical freedom for traditional bipolar devices and improves the view and trajectory to more lateral structures like the PPF and ITF. 3 Gross total resection was achieved without complications. The patient was discharged home with a partial V2 numbness (right superior gum) that improved with time. The endoscopic endonasal approach is a safe and effective technique even for large JNA. A multidisciplinary team consisting of an interventional radiologist, a skull base neurosurgeon, and an otorhinolaryngologist with expertise in endoscopic surgery may play a role for optimal surgical results. The patient consented for the procedure and for the video production.

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Abstract Background Consensus in timing of radiotherapy is yet to be established in esthesioneuroblastoma (ENB). Objective This study was aimed to investigate if planned adjuvant radiotherapy improves tumor control after complete margin negative resection of low Hyams' grade (1 or 2) ENB. Methods A retrospective review of patients with pathologically confirmed negative margin resection of Kadish's stage B or C and Hyams' grade 1 and 2 ENBs was conducted. Seventeen patients meeting the criteria were divided into the following two groups for cohort study: (1) those who underwent planned immediate postoperative adjuvant radiotherapy (IR group) and (2) those who did not (delayed radiotherapy [DR] group). Results The IR group included nine patients (Kadish's stage B in one and stage C in eight; Hyams' grade 1 in two and grade 2 in seven). Mean follow-up was 140.8 months. Seven patients (78%) had disease progression (DP) at a median of 88 months (four with cervical lymph node metastasis [CLNM], one with distant metastasis, and two with both local recurrence and CLNM). One patient experienced frontal lobe abscess. The DR group included eight patients (Kadish's stage B in six and stage C in two; all Hyams' grade 2). Mean follow-up was 123.3 months. Four (50%) patients who developed DP (all local recurrence) were salvaged with surgery and adjuvant radiotherapy at a median of 37.5 months. There was no statistically significant difference in DP rate (p = 0.23), time to DP (p = 0.26), or the local tumor control rate (p = 0.23). Conclusion In our limited cohort, immediate postoperative radiotherapy did not demonstrate superiority in tumor control, although risk of radiotherapy toxicity appears low.

AbstractObjective The objective of this study is to describe the clinical presentation, tumor characteristics, natural history, and treatment patterns of sinonasal osteosarcoma.Methods Fourteen patients who had been treated for osteosarcoma of the nasal cavity and paranasal sinuses at a tertiary care center were reviewed. In addition, a systematic review of the literature for osteosarcoma of the sinonasal cavity was performed.Results In a systematic review, including 14 patients from the authors' institution, 53 total studies including 88 patients were assessed. Median follow-up was 18 months (interquartile range: 8–39 months). The most common presenting symptoms were facial mass or swelling (34%), and nasal obstruction (30%). The most common paranasal sinus involved by tumor was the maxillary sinus (64%), followed by the ethmoid sinuses (52%). The orbit (33%), dura (13%) and infratemporal fossa (10%) were the most common sites of local invasion. The majority of patients underwent surgery followed by adjuvant therapy (52.4%). Increasing age was associated with decreased overall survival rate (unit risk ratio [95% confidence interval (CI)] = 1.02 [1.003–1.043]; p = 0.0216) and T4 disease was associated with decreased disease-specific survival rate (hazard ratio [HR] = 2.87; p = 0.0495). The 2- and 5-year overall survival rates were 68 and 40%, respectively, while 2- and 5-year disease-specific survival rates were 71% and 44%, respectively.Conclusion Sinonasal osteosarcomas are uncommon tumors and can pose a significant therapeutic challenge. Increasing age and T4 disease are associated with worse prognosis. This disease usually warrants consultation by a multidisciplinary team and consideration of multimodality therapy.

AbstractEsthesioneuroblastoma (ENB) is a rare olfactory malignancy that can present with locally advanced disease. At our institution, patients with ENB in whom the treating surgeon believes that a margin-negative resection is initially not achievable are selected to undergo induction with chemotherapy with or without radiotherapy prior to surgery. In a retrospective review of 61 patient records, we identified six patients (10%) treated with this approach. Five of six patients (83%) went on to definitive surgery. Prior to surgery, three of five patients (60%) had a partial response after induction therapy, whereas two of five (40%) had stable disease. Microscopically margin-negative resection was achieved in four of five (80%) of the patients who went on to surgery, while one patient had negative margins on frozen section but microscopically positive margins on permanent section. Three of five patients (60%) recurred after surgery; two of these patients died with recurrent/metastatic ENB. In summary, induction therapy may facilitate margin-negative resection in locally advanced ENB. Given the apparent sensitivity of ENB to chemotherapy and radiotherapy, future prospective studies should investigate the optimal multidisciplinary approach to improve long-term survival in this rare disease.