Outcomes of surgical revascularization for pediatric moyamoya disease and syndrome.

PURPOSE: Moyamoya disease and syndrome represent rare entities characterized by progressive stenosis and/or occlusion of the intracranial blood vessels. We present our series of patients with moyamoya disease and syndrome stratified by underlying disease and analyze differences in presentation and outcome following surgical revascularization. METHODS: This was an Institutional Review Board (IRB) approved, retrospective review of all patients surgically revascularized by the senior author (SNM) while at Children's National Hospital in Washington, DC. Demographic data, presenting symptoms and severity, surgical details, and functional and radiographic outcomes were obtained and analyzed for differences among the underlying cohorts of moyamoya disease and syndrome as well as by unilateral or bilateral disease and index or non-index surgeries. RESULTS: Twenty-two patients were identified with the following underlying diseases: six with idiopathic moyamoya disease, six with sickle cell anemia, five with trisomy 21, and five with neurofibromatosis type 1. Thirty hemispheres were revascularized with a significantly reduced rate of stroke from 3.19 strokes/year (SD = 3.10) to 0.13 strokes/year (SD = 0.25), p = 0.03. When analyzed by underlying cause of moyamoya syndrome, patients with neurofibromatosis type 1 were found to be significantly less likely than the other subtypes of moyamoya syndrome to have had either a clinical stroke (0.0% neurofibromatosis type 1 vs. 100.0% sickle cell, 60.0% trisomy 21, or 83.3% moyamoya disease, p < 0.01) or radiographic stroke (0.0% neurofibromatosis type 1 vs. 100.0% sickle cell, 60.0% trisomy 21, or 83.3% moyamoya disease, p < 0.01) at time of presentation. Patients with moyamoya syndrome associated with sickle cell disease were more likely to present with clinical and radiographic strokes. Additionally, patients with bilateral disease demonstrated no difference in final functional outcome compared to patients with unilateral disease (mRS 0.73 (SD = 1.33) vs. 1.29 (SD = 1.60), p = 0.63). CONCLUSION: Indirect surgical revascularization decreases stroke risk for pediatric patients with different forms of moyamoya disease and moyamoya syndrome. Additionally, these data suggest that sickle cell anemia-associated moyamoya syndrome may represent a more aggressive variant, while neurofibromatosis type 1 may represent a more benign variant.

Epidemiology of cranial infections in battlefield-related penetrating and open cranial injuries.

BACKGROUND: Penetrating brain injuries are a potentially lethal injury associated with substantial morbidity and mortality. We examined characteristics and outcomes among military personnel who sustained battlefield-related open and penetrating cranial injuries during military conflicts in Iraq and Afghanistan. METHODS: Military personnel wounded during deployment (2009-2014) were included if they sustained an open or penetrating cranial injury and were admitted to participating hospitals in the United States. Injury characteristics, treatment course, neurosurgical interventions, antibiotic use, and infection profiles were examined. RESULTS: The study population included 106 wounded personnel, of whom 12 (11.3%) had an intracranial infection. Posttrauma prophylactic antibiotics were prescribed in more than 98% of patients. Patients who developed central nervous system (CNS) infections were more likely to have undergone a ventriculostomy ( p = 0.003), had a ventriculostomy in place for a longer period (17 vs. 11 days; p = 0.007), had more neurosurgical procedures ( p < 0.001), and have lower presenting Glasgow Coma Scale ( p = 0.01) and higher Sequential Organ Failure Assessment scores ( p = 0.018). Time to diagnosis of CNS infection was a median of 12 days postinjury (interquartile range, 7-22 days) with differences in timing by injury severity (critical head injury had median of 6 days, while maximal [currently untreatable] head injury had a median of 13.5 days), presence of other injury profiles in addition to head/face/neck (median, 22 days), and the presence of other infections in addition to CNS infections (median, 13.5 days). The overall length of hospitalization was a median of 50 days, and two patients died. CONCLUSION: Approximately 11% of wounded military personnel with open and penetrating cranial injuries developed CNS infections. These patients were more critically injured (e.g., lower Glasgow Coma Scale and higher Sequential Organ Failure Assessment scores) and required more invasive neurosurgical procedures. LEVEL OF EVIDENCE: Prognostic and Epidemiological; Level IV.

Nonoperative management of enlarging syringomyelia in clinically stable patients after decompression of Chiari malformation type I.

OBJECTIVE: The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I). METHODS: A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. The management of these patients and their clinical courses were then described. RESULTS: A total of 153 children with CM-I and syringomyelia were evaluated between 2009 and 2017. Of these, 115 (68.8%) patients underwent surgical intervention: 40 patients underwent posterior fossa decompression (PFD) alone, 43 underwent PFD with duraplasty, and 32 underwent PFD with duraplasty and fourth ventricle stent placement. Eleven (7.19%) patients had increased syringomyelia on subsequent postoperative imaging. Three of these patients underwent revision surgery because of worsening scoliosis or pain, 2 of whom were lost to follow-up, and 4 were managed nonoperatively with close surveillance and serial MRI evaluations. The syringes decreased in size in 3 patients and resolved completely in 1 patient. CONCLUSIONS: Persistent or worsened syringomyelia after CM-I decompression is uncommon. In the absence of symptoms, nonoperative management with close observation is safe for patients with persistent syrinx.

Efficacy of Early (≤ 24 Hours), Late (25-72 Hours), and Delayed (>72 Hours) Surgery with Magnetic Resonance Imaging-Confirmed Decompression in American Spinal Injury Association Impairment Scale Grades C and D Acute Traumatic Central Cord Syndrome Caused by Spinal Stenosis.

The therapeutic significance of timing of decompression in acute traumatic central cord syndrome (ATCCS) caused by spinal stenosis remains unsettled. We retrospectively examined a homogenous cohort of patients with ATCCS and magnetic resonance imaging (MRI) evidence of post-treatment spinal cord decompression to determine whether timing of decompression played a significant role in American Spinal Injury Association (ASIA) motor score (AMS) 6 months following trauma. We used the t test, analysis of variance, Pearson correlation coefficient, and multiple regression for statistical analysis. During a 19-year period, 101 patients with ATCCS, admission ASIA Impairment Scale (AIS) grades C and D, and an admission AMS of ≤95 were surgically decompressed. Twenty-four of 101 patients had an AIS grade C injury. Eighty-two patients were males, the mean age of patients was 57.9 years, and 69 patients had had a fall. AMS at admission was 68.3 (standard deviation [SD] 23.4); upper extremities (UE) 28.6 (SD 14.7), and lower extremities (LE) 41.0 (SD 12.7). AMS at the latest follow-up was 93.1 (SD 12.8), UE 45.4 (SD 7.6), and LE 47.9 (SD 6.6). Mean number of stenotic segments was 2.8, mean canal compromise was 38.6% (SD 8.7%), and mean intramedullary lesion length (IMLL) was 23 mm (SD 11). Thirty-six of 101 patients had decompression within 24 h, 38 patients had decompression between 25 and 72 h, and 27 patients had decompression >72 h after injury. Demographics, etiology, AMS, AIS grade, morphometry, lesion length, surgical technique, steroid protocol, and follow-up AMS were not statistically different between groups treated at different times. We analyzed the effect size of timing of decompression categorically and in a continuous fashion. There was no significant effect of the timing of decompression on follow-up AMS. Only AMS at admission determined AMS at follow-up (coefficient = 0.31; 95% confidence interval [CI]:0.21; p = 0.001). We conclude that timing of decompression in ATCCS caused by spinal stenosis has little bearing on ultimate AMS at follow-up.

An Endovascular Surgery Experience in Far-Forward Military Healthcare-A Case Series.

INTRODUCTION: The advancement of interventional neuroradiology has drastically altered the treatment of stroke and trauma patients. These advancements in first-world hospitals, however, have rarely reached far forward military hospitals due to limitations in expertise and equipment. In an established role III military hospital though, these life-saving procedures can become an important tool in trauma care. MATERIALS AND METHODS: We report a retrospective series of far-forward endovascular cases performed by 2 deployed dual-trained neurosurgeons at the role III hospital in Kandahar, Afghanistan during 2013 and 2017 as part of Operations Resolute Support and Enduring Freedom. RESULTS: A total of 15 patients were identified with ages ranging from 5 to 42 years old. Cases included 13 diagnostic cerebral angiograms, 2 extremity angiograms and interventions, 1 aortogram and pelvic angiogram, 1 bilateral embolization of internal iliac arteries, 1 lingual artery embolization, 1 administration of intra-arterial thrombolytic, and 2 mechanical thrombectomies for acute ischemic stroke. There were no complications from the procedures. Both embolizations resulted in hemorrhage control, and 1 of 2 stroke interventions resulted in the improvement of the NIH stroke scale. CONCLUSIONS: Interventional neuroradiology can fill an important role in military far forward care as these providers can treat both traumatic and atraumatic cerebral and extracranial vascular injuries. In addition, knowledge and skill with vascular access and general interventional radiology principles can be used to aid in other lifesaving interventions. As interventional equipment becomes more available and portable, this relatively young specialty can alter the treatment for servicemen and women who are injured downrange.

Outcomes in Patients with Moyamoya Syndrome and Sickle Cell Disease: A Systematic Review.

BACKGROUND: Moyamoya syndrome, a progressive, idiopathic stenosis of the internal carotid arteries, results in increased risk for both ischemic and hemorrhagic strokes. Revascularization procedures have been shown in small studies to be both safe and efficacious for these patients; however, randomized controlled trials are lacking. The goal of this systematic review is to organize the literature evaluating surgical intervention versus conservative medical management. METHODS: A systematic review was performed including studies with 3 or more participants with moyamoya syndrome in the setting of sickle cell disease and a measured outcome after either medical or surgical intervention. Relevant studies were identified using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria and a set of predetermined key words. RESULTS: Sixty-one articles were identified with 6 articles ultimately included in this review (N = 122). Of the patients, 73 (59.8%) were revascularized surgically (all indirect procedures), whereas 49 (40.2%) remained on chronic transfusion therapy. Of the patients that underwent indirect revascularization surgery, a total of 1 perioperative (1.4%) and 4 postoperative strokes (5.5%) were reported over 44 months (1 stroke per 53.3 patient-years). In comparison, an average of 46.5% of patients who were receiving chronic transfusions had major events (stroke or transient ischemic attack) while undergoing therapy (1 stroke per 13.65 patient-years, P = 0.00215). CONCLUSIONS: We present a large systematic review of the literature regarding outcomes of surgical and medical management for patients with moyamoya syndrome and sickle cell disease. The findings redemonstrate the efficacy and safety of surgical revascularization, and advocate for earlier discussion around surgical intervention.

Return-to-active-duty rates after anterior cervical spine surgery in military pilots.

OBJECTIVESymptomatic cervical spondylosis with or without radiculopathy can ground an active-duty military pilot if left untreated. Surgically treated cervical spondylosis may be a waiverable condition and allow return to flying status, but a waiver is based on expert opinion and not on recent published data. Previous studies on rates of return to active duty status following anterior cervical spine surgery have not differentiated these rates among military specialty occupations. No studies to date have documented the successful return of US military active-duty pilots who have undergone anterior cervical spine surgery with cervical fusion, disc replacement, or a combination of the two. The aim of this study was to identify the rate of return to an active duty flight status among US military pilots who had undergone anterior cervical discectomy and fusion (ACDF) or total disc replacement (TDR) for symptomatic cervical spondylosis.METHODSThe authors performed a single-center retrospective review of all active duty pilots who had undergone either ACDF or TDR at a military hospital between January 2010 and June 2017. Descriptive statistics were calculated for both groups to evaluate demographics with specific attention to preoperative flight stats, days to recommended clearance by neurosurgery, and days to return to active duty flight status.RESULTSAuthors identified a total of 812 cases of anterior cervical surgery performed between January 1, 2010, and June 1, 2017, among active duty, reserves, dependents, and Department of Defense/Veterans Affairs patients. There were 581 ACDFs and 231 TDRs. After screening for military occupation and active duty status, there were a total of 22 active duty pilots, among whom were 4 ACDFs, 17 TDRs, and 2 hybrid constructs. One patient required a second surgery. Six (27.3%) of the 22 pilots were nearing the end of their career and electively retired within a year of surgery. Of the remaining 16 pilots, 11 (68.8%) returned to active duty flying status. The average time to be released by the neurosurgeon was 128 days, and the time to return to flying was 287 days. The average follow-up period was 12.3 months.CONCLUSIONSAdhering to military service-specific waiver guidelines, military pilots may return to active duty flight status after undergoing ACDF or TDR for symptomatic cervical spondylosis.

Isolated Transverse Process Spinal Fractures Increase the Likelihood of Incurring Visceral and Pelvic Injuries: A Retrospective Review at a Level-1 Trauma Center.

BACKGROUND: Although isolated transverse process fractures (ITPF) do not confer any inherent risk of compromised spinal stability, there is increasing interest in their overall prognostic significance. As a proxy for localized or directional forces in high-energy traumatic mechanisms, ITPF may serve as an indicator for the presence of other coexisting traumatic injuries. Specific injuries may be predicted by the presence of ITPF at specific spinal levels, but few studies have examined this in depth and may not account for confounding variables. METHODS: We retrospectively analyzed data from 306 patients presenting with acute traumatic ITPF. ITPF number and location by spinal segment were determined from initial computed tomography. Mechanism of trauma, Injury Severity Score, and extent of non-spinal-associated injuries were recorded. Correlation analysis compared ITPF location with injury severity, non-spinal-associated injury location, type, and patterns. Significant injury associations were further explored with logistic regression analysis controlling for age, mechanism of injury, and Injury Severity Score. RESULTS: The adjusted odds of pulmonary visceral injury was 4.69 (95% confidence interval, 2.33-9.44) times higher among patients with thoracic-level ITPF compared with other ITPF levels. Lumbar ITPFs had increased odds of abdominal visceral injury (odds ratio, 4.85; P = 0.0002), pelvic fractures (odds ratio, 4.2; P < 0.0001). The number needed to scan to observe a pelvic injury among patients with lumbar ITPF was 3. Other significant associations were also observed. CONCLUSIONS: Spinal level of ITPF is associated with increased likelihood of specific patterns of injury, and additional investigation is warranted.

Degree of Vascular Encasement in Sphenoid Wing Meningiomas Predicts Postoperative Ischemic Complications.

BACKGROUND: Sphenoid wing meningiomas (SWMs) can encase arteries of the circle of Willis, increasing their susceptibility to intraoperative vascular injury and severe ischemic complications. OBJECTIVE: To demonstrate the effect of circumferential vascular encasement in SWM on postoperative ischemia. METHODS: A retrospective review of 75 patients surgically treated for SWM from 2009 to 2015 was undertaken to determine the degree of circumferential vascular encasement (0°-360°) as assessed by preoperative magnetic resonance imaging (MRI). A novel grading system describing "maximum" and "total" arterial encasement scores was created. Postoperative MRIs were reviewed for total ischemia volume measured on sequential diffusion-weighted images. RESULTS: Of the 75 patients, 89.3% had some degree of vascular involvement with a median maximum encasement score of 3.0 (2.0-3.0) in the internal carotid artery (ICA), M1, M2, and A1 segments; 76% of patients had some degree of ischemia with median infarct volume of 3.75 cm 3 (0.81-9.3 cm 3 ). Univariate analysis determined risk factors associated with larger infarction volume, which were encasement of the supraclinoid ICA ( P < .001), M1 segment ( P < .001), A1 segment ( P = .015), and diabetes ( P = .019). As the maximum encasement score increased from 1 to 5 in each of the significant arterial segments, so did mean and median infarction volume ( P < .001). Risk for devastating ischemic injury >62 cm 3 was found when the ICA, M1, and A1 vessels all had ≥360° involvement ( P = .001). Residual tumor was associated with smaller infarct volumes ( P = .022). As infarction volume increased, so did modified Rankin Score at discharge ( P = .025). CONCLUSION: Subtotal resection should be considered in SWM with significant vascular encasement of proximal arteries to limit postoperative ischemic complications.