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Iulia Peciu-Florianu, Maximilien Vermandel, Nicolas Reyns and Constantin Tuleasca

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Daniel Blatt, Barry Cheaney II, Katherine Holste, Seshadri Balaji and Ahmed M. Raslan

OBJECTIVE

Congenital long QT syndrome (LQTS) provides an opportunity for neurosurgical intervention. Medication and implantable cardiac defibrillator (ICD)–refractory patients often require left cardiac sympathetic denervation (LCSD) via anterior video-assisted thoracoscopic surgery (VATS). However, this approach has major pulmonary contraindications and risks, with a common concern in children being their inability to tolerate single-lung ventilation. At Oregon Health & Science University, the authors have developed a posterior approach—extrapleural, minimally invasive, T1–5 LCSD—that minimizes this risk.

METHODS

A 9-year-old girl with LQTS type III presented to the emergency department while experiencing ventricular tachycardia (VT) and ventricular fibrillation (VF) with multiple ICD firings. Medical management failed to resolve the VF/VT. VATS was attempted but could not be safely performed due to respiratory insufficiency. The patient was reintubated for dual-lung ventilation and repositioned prone. Her respiratory insufficiency resolved. Using METRx serial dilating tubes under the microscope, the left T1–5 sympathetic ganglia were sectioned and removed.

RESULTS

Postoperatively, the patient had no episodes of VF/VT, pneumothorax, hemothorax, or Horner syndrome. With mexiletine and propranolol, she has remained largely VF/VT free, with only one VT episode during the 2-year follow-up period.

CONCLUSIONS

Minimally invasive, posterior, extrapleural, T1–5 LCSD is safe and effective for treating congenital LQTS in children, while minimizing the risks associated with VATS.

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Miner Ross, Priscilla S. Pang, Ahmed M. Raslan, Nathan R. Selden and Justin S. Cetas

OBJECTIVE

Conventional management of patients with neurotrauma frequently consists of routine, repeat head CT at preordained intervals with ICU-level monitoring, regardless of injury severity. The Brain Injury Guidelines (BIG) are a classification tool for stratifying patients into injury severity and risk-of-progression categories based on presenting clinical and radiographic findings. In the present study, the authors aimed to validate BIG criteria at a single level 1 trauma center.

METHODS

Patients were classified according to BIG criteria and evaluated for subsequent radiographic progression or development of neurological decline. A 2-year retrospective cohort review of consecutive patients with neurotrauma (n = 590) was undertaken. The authors then developed a modified BIG algorithm for use at their institution and followed its implementation prospectively over 555 consecutive patients.

RESULTS

In the retrospective analysis, no patient in the BIG 1 category (n = 88, 14.9%) demonstrated progression or neurological decline, and 7.5% of BIG 2 patients (n = 107, 18.1%) demonstrated mild radiographic progression without any decline or need for additional neurosurgical or medical intervention, whereas 15.4% of BIG 3 patients (n = 395, 66.9%) underwent additional neurosurgical procedures. In the prospective analysis, no BIG 1 (n = 105, 18.9%) or BIG 2 (n = 48, 8.6%) patients demonstrated a clinical decline or required any further neurosurgical intervention. By contrast, 12.9% of BIG 3 patients (n = 402, 72%) required immediate neurosurgical intervention, and a further 2.0% required delayed intervention based on clinical and/or radiographic evidence of injury progression.

CONCLUSIONS

Application of the BIG criteria in a single large level 1 trauma center reliably sorted patients into appropriate risk categories that accurately guided ongoing management.

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Zoe E. Teton, Daniel Blatt, Katherine Holste, Ahmed M. Raslan and Kim J. Burchiel

OBJECTIVE

Hemifacial spasm (HFS), largely caused by neurovascular compression (NVC) of the facial nerve, is a rare condition characterized by paroxysmal, unilateral, involuntary contraction of facial muscles. It has long been suggested that these symptoms are due to compression at the transition zone of the facial nerve. The aim of this study was to examine symptom-free survival and long-term quality of life (QOL) in HFS patients who underwent microvascular decompression (MVD). A secondary aim was to examine the benefit of utilizing fused MRI and MRA post hoc 3D reconstructions to better characterize compression location at the facial nerve root exit zone (fREZ).

METHODS

The authors retrospectively analyzed patients with HFS who underwent MVD at a single institution, combined with a modified HFS-7 telephone questionnaire. Kaplan-Meier analysis was used to determine event-free survival, and the Wilcoxon signed-rank test was used to compare pre- and postoperative HFS-7 scores.

RESULTS

Thirty-five patients underwent MVD for HFS between 2002 and 2018 with subsequent 3D reconstructions of preoperative images. The telephone questionnaire response rate was 71% (25/35). If patients could not be reached by telephone, then the last clinic follow-up date was recorded and any recurrence noted. Twenty-four patients (69%) were symptom free at longest follow-up. The mean length of follow-up was 2.4 years (1 month to 8 years). The mean symptom-free survival time was 44.9 ± 5.8 months, and the average symptom-control survival was 69.1 ± 4.9 months. Four patients (11%) experienced full recurrence. Median HFS-7 scores were reduced by 18 points after surgery (Z = −4.013, p < 0.0001). Three-dimensional reconstructed images demonstrated that NVC most commonly occurred at the attached segment (74%, 26/35) of the facial nerve within the fREZ and least commonly occurred at the traditionally implicated transition zone (6%, 2/35).

CONCLUSIONS

MVD is a safe and effective treatment that significantly improves QOL measures for patients with HFS. The vast majority of patients (31/35, 89%) were symptom free or reported only mild symptoms at longest follow-up. Symptom recurrence, if it occurred, was within the first 2 years of surgery, which has important implications for patient expectations and informed consent. Three-dimensional image reconstruction analysis determined that culprit compression most commonly occurs proximally along the brainstem at the attached segment. The success of this procedure is dependent on recognizing this pattern and decompressing appropriately. Three-dimensional reconstructions were found to provide much clearer characterization of this area than traditional preoperative imaging. Therefore, the authors suggest that use of these reconstructions in the preoperative setting has the potential to help identify appropriate surgical candidates, guide operative planning, and thus improve outcome in patients with HFS.

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Diana Ko, Daniel Blatt, Chafic Karam, Kunal Gupta and Ahmed M. Raslan

Nusinersen (Spinraza) is a US Food and Drug Administration–approved intrathecal medication for the treatment of spinal muscular atrophy (SMA). Adult patients with SMA often undergo thoracolumbar fusion to treat neurogenic scoliosis, preventing thecal access. The authors report a laminotomy technique and the ease of intrathecal access in three SMA patients with prior thoracolumbar fusions.

Patients were positioned in the lateral decubitus position or prone. Lumbar laminotomy was performed below the conus, between the lateral longitudinal rods, to preserve mechanical stability. Fluoroscopy provided real-time identification of instruments. Hardware was contoured with a carbide drill bit to develop the surgical window. Fiducial screws were placed along the perimeter for demarcation. Sublaminar wire removal caused dural defects that were repaired with a layer of dural substitute onlay and sealant. All patients successfully received nusinersen thecal injections via lumbar puncture by an interventional radiologist. Fluoroscopy time ranged from 6 to 36 seconds. No postoperative pseudomeningoceles, cerebrospinal fluid leaks, or wound complications occurred.

For patients with SMA and posterior fusion from prior scoliosis treatment, lumbar laminotomy is an effective method for creating thecal access for the administration of nusinersen.

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Zoe E. Teton, Daniel Blatt, Amr AlBakry, James Obayashi, Gulsah Ozturk, Vural Hamzaoglu, Philippe Magown, Nathan R. Selden, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

Despite rapid development and expansion of neuromodulation technologies, knowledge about device and/or therapy durability remains limited. The aim of this study was to evaluate the long-term rate of hardware and therapeutic failure of implanted devices for several neuromodulation therapies.

METHODS

The authors performed a retrospective analysis of patients’ device and therapy survival data (Kaplan-Meier survival analysis) for deep brain stimulation (DBS), vagus nerve stimulation (VNS), and spinal cord stimulation (SCS) at a single institution (years 1994–2015).

RESULTS

During the study period, 450 patients underwent DBS, 383 VNS, and 128 SCS. For DBS, the 5- and 10-year initial device survival was 87% and 73%, respectively, and therapy survival was 96% and 91%, respectively. For VNS, the 5- and 10-year initial device survival was 90% and 70%, respectively, and therapy survival was 99% and 97%, respectively. For SCS, the 5- and 10-year initial device survival was 50% and 34%, respectively, and therapy survival was 74% and 56%, respectively. The average initial device survival for DBS, VNS, and SCS was 14 years, 14 years, and 8 years while mean therapy survival was 18 years, 18 years, and 12.5 years, respectively.

CONCLUSIONS

The authors report, for the first time, comparative device and therapy survival rates out to 15 years for large cohorts of DBS, VNS, and SCS patients. Their results demonstrate higher device and therapy survival rates for DBS and VNS than for SCS. Hardware failures were more common among SCS patients, which may have played a role in the discontinuation of therapy. Higher therapy survival than device survival across all modalities indicates continued therapeutic benefit beyond initial device failures, which is important to emphasize when counseling patients.

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Contemporary concepts of pain surgery

JNSPG 75th Anniversary Invited Review Article

Kim J. Burchiel and Ahmed M. Raslan

Pain surgery is one of the historic foundations of neurological surgery. The authors present a review of contemporary concepts in surgical pain management, with reference to past successes and failures, what has been learned as a subspecialty over the past 50 years, as well as a vision for current and future practice. This subspecialty confronts problems of cancer pain, nociceptive pain, and neuropathic pain. For noncancer pain, ablative procedures such as dorsal root entry zone lesions and rhizolysis for trigeminal neuralgia (TN) should continue to be practiced. Other procedures, such as medial thalamotomy, have not been proven effective and require continued study. Dorsal rhizotomy, dorsal root ganglionectomy, and neurotomy should probably be abandoned. For cancer pain, cordotomy is an important and underutilized method for pain control. Intrathecal opiate administration via an implantable system remains an important option for cancer pain management. While there are encouraging results in small case series, cingulotomy, hypophysectomy, and mesencephalotomy deserve further detailed analysis. Electrical neuromodulation is a rapidly changing discipline, and new methods such as high-frequency spinal cord stimulation (SCS), burst SCS, and dorsal root ganglion stimulation may or may not prove to be more effective than conventional SCS. Despite a history of failure, deep brain stimulation for pain may yet prove to be an effective therapy for specific pain conditions. Peripheral nerve stimulation for conditions such as occipital neuralgia and trigeminal neuropathic pain remains an option, although the quality of outcomes data is a challenge to these applications. Based on the evidence, motor cortex stimulation should be abandoned. TN is a mainstay of the surgical treatment of pain, particularly as new evidence and insights into TN emerge. Pain surgery will continue to build on this heritage, and restorative procedures will likely find a role in the armamentarium. The challenge for the future will be to acquire higher-level evidence to support the practice of surgical pain management.

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Fran A. Hardaway, Katherine Holste, Gulsah Ozturk, David Pettersson, Jeffrey M. Pollock, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

The pathophysiology of trigeminal neuralgia (TN) in patients without neurovascular compression (NVC) is not completely understood. The objective of this retrospective study was to evaluate the hypothesis that TN patients without NVC differ from TN patient with NVC with respect to brain anatomy and demographic characteristics.

METHODS

Six anatomical brain measurements from high-resolution brain MR images were tabulated; anterior-posterior (AP) prepontine cistern length, cerebellopontine angle (CPA) cistern volume, nerve-to-nerve distance, symptomatic nerve length, pons volume, and posterior fossa volume were assessed on OsiriX. Brain MRI anatomical measurements from 232 patients with either TN type 1 or TN type 2 (TN group) were compared with measurements obtained in 100 age- and sex-matched healthy controls (control group). Two-way ANOVA tests were conducted on the 6 measurements relative to group and NVC status. Bonferroni adjustments were used to correct for multiple comparisons. A nonhierarchical k-means cluster analysis was performed on the TN group using age and posterior fossa volume as independent variables.

RESULTS

Within the TN group, females were found to be younger than males and less likely to have NVC. The odds ratio (OR) of females not having NVC compared to males was 2.7 (95% CI 1.3–5.5, p = 0.017). Patients younger than 30 years were much less likely to have NVC compared to older patients (OR 4.9, 95% CI 1.3–18.4, p = 0.017). The mean AP prepontine cistern length and symptomatic nerve length were smaller in the TN group than in the control group (5.3 vs 6.5 mm and 8.7 vs 9.7 mm, respectively; p < 0.001). The posterior fossa volume was significantly smaller in TN patients without NVC compared to those with NVC. A TN group cluster analysis suggested a sex-dependent difference that was not observed in those without NVC. Factorial ANOVA and post hoc testing found that findings in males without NVC were significantly different from those in controls or male TN patients with NVC and similar to those in females (female controls as well as female TN patients with or without NVC).

CONCLUSIONS

Posterior fossa volume in males was larger than posterior fossa volume in females. This finding, along with the higher incidence of TN in females, suggests that smaller posterior fossa volume might be an independent factor in the pathophysiology of TN, which warrants further study.

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Fran A. Hardaway, Hanna C. Gustafsson, Katherine Holste, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

Pain relief following microvascular decompression (MVD) for trigeminal neuralgia (TN) may be related to pain type, degree of neurovascular conflict, arterial compression, and location of compression. The objective of this study was to construct a predictive pain-free scoring system based on clinical and radiographic factors that can be used to preoperatively prognosticate long-term outcomes for TN patients following surgical intervention (MVD or internal neurolysis [IN]). It was hypothesized that contributing factors would include pain type, presence of an artery or vein, neurovascular conflict severity, and compression location (root entry zone).

METHODS

At the authors’ institution 275 patients with type 1 or type 2 TN (TN1 or TN2) underwent MVD or IN following preoperative high-resolution brain MRI studies. Outcome data were obtained retrospectively by chart review and/or phone follow-up. Characteristics of neurovascular conflict were obtained from preoperative MRI studies. Factors that resulted in a probability value of < 0.05 on univariate logistic regression analyses were entered into a multivariate Cox regression analysis in a backward stepwise fashion. For the multivariate analysis, significance at the 0.15 level was used. A prognostic system was then devised with 4 possible scores (0, 1, 2, or 3) and pain-free survival analyses conducted.

RESULTS

Univariate predictors of pain-free survival were pain type (p = 0.013), presence of any vessel (p = 0.042), and neurovascular compression severity (p = 0.038). Scores of 0, 1, 2, and 3 were found to be significantly different in regard to pain-free survival (log rank, p = 0.005). At 5 and 10 years there were 36%, 43%, 61%, and 69%, and 36%, 43%, 56%, and 67% pain-free survival rates in groups 0, 1, 2, and 3, respectively. While TN2 patients had worse outcomes regardless of score, a subgroup analysis of TN1 patients with higher neurovascular conflict (score of 3) had significantly better outcomes than TN1 patients without severe neurovascular conflict (score of 1) (log rank, p = 0.005). Regardless of pain type, those patients with severe neurovascular conflict were more likely to have arterial compression (99%) compared to those with low neurovascular conflict (p < 0.001).

CONCLUSIONS

Pain-free survival was predicted by a scoring system based on preoperative clinical and radiographic findings. Higher scores predicted significantly better pain relief than lower scores. TN1 patients with severe neurovascular conflict had the best long-term pain-free outcome.