Faculty Bibliography

OBJECTIVE:The choice of transsylvian versus transcortical corridors for resection of insular gliomas remains controversial. Functional pathway compromise from transcortical transgression and vascular injury during transsylvian dissection are the primary concerns. In this study, data from a single-center experience with both approaches were compared to determine whether one approach was associated with a higher rate of morbidity than the other. METHODS:The authors identified 100 consecutive patients who underwent resection of pure insular gliomas at the Barrow Neurological Institute. Volumetric analysis was performed using FLAIR and contrast-enhanced T1-weighted MRI for low- and high-grade gliomas, respectively, for extent of resection (EOR) and diffusion-weighted sequences were used to detect for postoperative ischemia. Step-wise logistic regression analysis was performed to identify predictors of neurological morbidity. RESULTS:Data from 100 patients with low-grade or high-grade insular gliomas were analyzed. Fifty-two patients (52%) underwent a transsylvian approach, and 48 patients (48%) underwent a transcortical approach. The mean (± SD) EOR was 91.6% ± 12.4% in the transsylvian group and 88.6% ± 14.2% in the transcortical group (p = 0.26). Clinical outcome metrics for the 2 groups were similar. Overall, 13 patients (25%) in the transsylvian group and 10 patients (21%) in the transcortical group had evidence of ischemia on postoperative MR images. For both approaches, high-grade histology was associated with permanent morbidity (p = 0.01). For patients with gliomas located within the superior-posterior quadrant of the insula, development of postoperative ischemia was associated with only the transsylvian approach (46% vs 0%, p = 0.02). CONCLUSIONS:Areas of restricted diffusion are common on postoperative MRI following resection of insular gliomas, but only a minority of these patients develop permanent neurological deficits. Insular glioma patients with high-grade histology may be at particular risk for developing symptomatic postoperative ischemia. Both the transcortical and transsylvian corridors are associated with reasonable morbidity profiles, although gliomas situated within the superior-posterior quadrant of the insula are more safely accessed with a transcortical approach.

OBJECTIVE:The Barrow Innovation Center comprises an educational program in medical innovation that enables residents to identify problems in patient care and rapidly develop and implement solutions to these problems. Residents involved in this program noted an elevated risk of iatrogenic spinal cord injury during posterior cervical and thoracic procedures. The objective of this study was to describe this complication, and a novel solution was developed through a new innovation training program. METHODS:A case report demonstrates the risk of iatrogenic spinal cord injury during posterior cervical decompression and fusion. Solutions to this problem were developed at the innovation center via an iterative process of prototype creation, cadaveric testing, and redesign. Patent law students who partnered with the center wrote and filed a provisional patent protecting the novel prototype designs. RESULTS:The concept of a protective shield for the spinal cord was developed, and within only 6 weeks the devices were provisionally patented and used in the operating room. This device was named the Myeloshield. Initial clinical experience indicates that the Myeloshield can be used without impeding the flow of surgery and has the potential to prevent iatrogenic spinal cord injury; this experience is presented through 2 case reports demonstrating the use of Myeloshields in the operating room. CONCLUSIONS:This report demonstrates how programs like the Barrow Innovation Center can provide neurosurgery residents with a unique educational experience in medical device innovation and intellectual property development and can serve as an avenue of surgical quality improvement and problem solving.

OBJECTIVE:The objective of this study was to assess long-term outcomes of facial pain and numbness after radiosurgery for multiple sclerosis (MS)-related trigeminal neuralgia (MS-TN). METHODS:The authors conducted a retrospective review of their Gamma Knife radiosurgeries (GKRSs) to identify all patients treated for MS-TN (1998-2014) with at least 3 years of follow-up. Treatment and clinical data were obtained via chart review and mailed or telephone surveys. Pain control was defined as a facial pain score of I-IIIb on the Barrow Neurological Institute (BNI) Facial Pain Intensity Scale. Kaplan-Meier analysis was performed to determine the rates of pain control after index and first salvage GKRS procedures. Patients could have had more than 1 salvage procedure. Pain control rates were based on the number of patients at risk during follow-up. RESULTS:Of the 50 living patients who underwent GKRS, 42 responded to surveys (31 women [74%], median age 59 years, range 32-76 years). During the initial GKRS, the trigeminal nerve root entry zone was targeted with a single isocenter, using a 4-mm collimator with the 90% isodose line completely covering the trigeminal nerve and the 50% isodose line abutting the surface of the brainstem. The median maximum radiation dose was 85 Gy (range 50-85 Gy). The median follow-up period was 78 months (range 36-226 months). The rate of pain control after the index GKRS (n = 42) was 62%, 29%, 22%, and 13% at 1, 3, 5, and 7 years, respectively. Twenty-eight patients (67%) underwent salvage treatment, including 25 (60%) whose first salvage treatment was GKRS. The rate of pain control after the first salvage GKRS (n = 25) was 84%, 50%, 44%, and 17% at 1, 3, 5, and 7 years, respectively. The rate of pain control after the index GKRS with or without 1 salvage GKRS (n = 33) was 92%, 72%, 52%, 46%, and 17% at 1, 3, 5, 7, and 10 years, respectively. At last follow-up, 9 (21%) of the 42 patients had BNI grade I facial pain, 35 (83%) had achieved pain control, and 4 (10%) had BNI grade IV facial numbness (very bothersome in daily life). CONCLUSIONS:Index GKRS offers good short-term pain control for MS-TN, but long-term pain control is uncommon. If the index GKRS fails, salvage GKRS appears to offer beneficial pain control with low rates of bothersome facial numbness.

OBJECTIVE:Preoperative embolization may facilitate skull base meningioma resection, but its safety and efficacy in the Onyx era have not been investigated. In this retrospective cohort study, we evaluated the outcomes of preoperative embolization of skull base meningiomas using Onyx as the primary embolysate. METHODS:We queried an endovascular database for patients with skull base meningiomas who underwent preoperative embolization at our institution in 2007-2017. Patient, tumor, procedure, and outcome data were analyzed. RESULTS:. There were 1, 2, or 3 arterial pedicles embolized in 21 cases (75%), 6 cases (21%), and 1 case (4%), respectively. The embolized pedicles included branches of the middle meningeal artery in 19 cases (68%), the internal maxillary artery in 8 cases (29%), the ascending pharyngeal artery in 2 cases (7%), and the posterior auricular, ophthalmic, occipital, and anterior cerebral arteries in 1 case each (4%). The embolysates used were Onyx alone in 20 cases (71%), n-butyl cyanoacrylate alone in 3 cases (11%), coils/particles and Onyx/n-butyl cyanoacrylate in 2 cases each (7%), and Onyx and coils in 1 case (4%). The median degree of tumor devascularization was 60%. Significant neurologic morbidity occurred in 1 patient (4%) who developed symptomatic peritumoral edema after Onyx embolization. CONCLUSIONS:For appropriately selected skull base meningiomas supplied by dura mater-based arterial pedicles without distal cranial nerve supply, preoperative embolization with current embolysate technology affords substantial tumor devascularization with a low complication rate.

This corrects the article DOI: 10.1038/ncomms14433.

Primary cardiomyopathy is one of the most common inherited cardiac diseases and harbors significant phenotypic and genetic heterogeneity. Because of this, genetic testing has become standard in treatment of this disease group. Indeed, in recent years, next-generation DNA sequencing has found broad applications in medicine, both as a routine diagnostic tool for genetic disorders and as a high-throughput discovery tool for identifying novel disease-causing genes. We describe a male infant with primary dilated cardiomyopathy who was diagnosed using intrauterine echocardiography and found to progress to hypertrophic cardiomyopathy after birth. This proband was born to a nonconsanguineous family with a past history of a male fetus that died because of cardiac abnormalities at 30 wk of gestation. Using whole-exome sequencing, a novel homozygous frameshift mutation (c.2018delC; p.Gln675SerfsX30) in ALPK3 was identified and confirmed with Sanger sequencing. Heterozygous family members were normal with echocardiographic examination. To date, only two studies have reported homozygous pathogenic variants of ALPK3, with a total of seven affected individuals with cardiomyopathy from four unrelated consanguineous families. We include a discussion of the patient's phenotypic features and a review of relevant literature findings.

Occlusion of the basilar artery can be treated with endovascular thrombectomy, although the results have not been well studied. Persistent fetal cerebrovascular anatomy can lead to unusual presentation of carotid atherosclerotic disease and can be a barrier to successful mechanical thrombectomy if not recognized. This case presents a rare persistent carotico-basilar anastomosis which resulted in basilar occlusion via the persistent hypoglossal artery and coincident absence of contralateral vertebral arterial access due to a left vertebral artery terminating in the left posterior inferior cerebellar artery. Preoperative recognition of this anatomy afforded by review of cross-sectional imaging was critical to success during this emergent procedure.