Cerebral and spinal cord high-flow arteriovenous fistulae (HFAVF) are part of the spectrum of lesions found in Hereditary Hemorrhagic Telangiectasia (HHT). HFAVF consist of communications between large arteries and veins without interposed nidi or capillary transitions. The association between HHT and cerebral or spinal HFAVF in children has been reported and suggested as a potential marker for HHT. We present a newborn with bilateral intracranial HFAVF tested positive for HHT1 and belonging to a family non known for carrying a HHT mutation. We also review reported cases of neonates and infants with cerebral and spinal HFAVF emphasizing their associations with genetic syndromes. Our aim is to add a new case to the pertinent literature and emphasize the need for molecular testing in children with spinal or brain HFAVF.

Background: In 2015 guidelines regarding endovascular treatment (ET) of Large Vessel Occlusion (LVO) in acute ischemic stroke (AIS) were changed, leading to more patients being transferred to comprehensive stroke centers (CSC) for ET in selected patients, sometimes bypassing primary stroke centers. In the era of ET, there is a need for a simple yet sensitive pre-hospital tool to triage appropriate patients to CSCs. Many prehospital stroke scales predicting LVO are not in widespread clinical use because they are complex and not reliable. A recently published Denmark study demonstrated the PASS tool (Score range 0-3) for detecting LVO where a score of ≥2 was considered to be optimal in predicting LVO with sensitivity of 0.66. Methods: A retrospective analysis of AIS patients with confirmed anterior circulation LVO by catheter-based cerebral angiography between January 2015 and June 2016 was conducted. PASS scores were calculated and correlated with NIHSS to assess for severity of the stroke. Results: Fifty-four patients received ET during the study period. Those who had posterior circulation LVO were excluded, leaving 44 patients for final analysis. Only 5 (11.4%) patients had PASS score of <2 while 39 patients (88.6 %) had a score of ≥2 showing sensitivity of 0.89 for those patients with LVO. Average NIHSS scores were 11 (95% CI 6.6-15) for PASS <2 and 20 (95% CI 18.5-22.5) for PASS ≥2 (p value 0.005). Conclusion: The PASS tool is simple, quick, and easy to perform and has high sensitivity in AIS patients with LVO. To assess its value and efficacy in real time it should be implemented into EMS systems and be performed in the pre-hospital setting.

Background: Prehospital triage tools are essential to identify large vessel occlusion (LVO) in order to triage patients to a comprehensive stroke center for timely endovascular treatment (ET). Prehospital Acute Stroke Severity Scale (PASS) (score range 0-3) was recently identified as a valuable tool to predict LVO. Several studies have shown that in patients treated with IV tPA, a score calculated by multiplying admission NIHSS by the time from symptom onset to tPA treatment (in hours) can predict outcome. In our study, we applied similar concept for patients with LVO who underwent successful ET. Methods: We retrospectively reviewed all LVO patients between January 2015 and June 2016 who received ET. We analyzed the association of time of symptom onset to groin time (OGT), NIHSS, PASS, NIHSS-OGT, and PASS-OGT with modified Rankin scale (mRS) at the time of discharge. Results: Fifty-four patients underwent ET during the study period. Patients with posterior circulation LVO and those treated after 6 hours from last known normal were excluded. A total of 34 patients were left for final analysis. Patients with a good outcome (mRS ≤2) had an average NIHSS-OGT score of 43.2 (95% CI: 29.7-56.8) and PASS-OGT score of 5.52 (95% CI: 4.48-6.56). Patient’s with poor to miserable outcomes (mRS 3-6) average NIHSS-OGT 84.7 (95% CI: 72.8-96.6) and PASS-OGT average 9.8 (95% CI: 8.3-11.2). For NIHSS-OGT cut off of 55 the sensitivity and specificity was 0.75 and 0.85 respectively; diagnostic odds ratio 16.5 (96% CI: 2.41-112.83). For PASS-OGT cut off of 6.5 the sensitivity and specificity were 0.88 and 0.76 respectively; diagnostic odds ratio 23.33 (95% CI: 2.37-229.33). The wide confidence intervals can be attributed to small sample size. Conclusion: Our study indicates NIHSS–OGT and PASS-OGT scores have a linear relationship with discharge mRS and can reliably predict early clinical outcomes after ET. Further confirmation with randomized control trials is needed.

Introduction: Large Vessel Occlusion (LVO) makes up about 30% of acute strokes. There are several published tools designed for detection of LVO for use in the prehospital setting. The ideal prehospital LVO tool should be simple and easy for prehospital staff to use and result in few LVO patients being missed as well as prevent over-testing and unnecessary transferring of patients without an LVO. Methods: From January 2015 to December 2016 1,108 acute ischemic stroke cases were identified; cases which did not present in the acute (<24hrs from onset) period and cases which did not have angiography (CTA, MRA, or catheter angiogram) performed were excluded, leaving 973 cases for analysis. The components of the initial NIHSS evaluation was utilized to calculate various prehospital LVO scales (PASS, VAN, RACE, FAST-ED, and CPSSS). Each chart was reviewed for the presence or absence of LVO defined as M1, M2, ICA or basilar occlusion. The relative performance of each of the prehospital LVO scales were compared using 2x2 analysis. Results: In the study population, 30% were LVO positive, 24% received IV tPA, and 9% received endovascular thrombectomy. Comparison of tools using previously defined cutoff points yielded the sensitivity and specificity characteristics depicted in table 1 which are compared to the NIH Stroke Scale cutoff point of 6 which has been previously defined as optimal for LVO detection. Conclusion: In this analysis, it is evident that there are distinct trade-offs, with no tool being superior when it comes to having both optimal sensitivity and specificity. Although the NIHSS is the most sensitive tool, use of this scale is not practical in the prehospital setting. Therefore, we support the use of simple tools such as PASS and VAN given the relative ease with which these tools can be learned and applied by EMS.

Introduction: Endovascular Therapy (ET) has become the new standard of care for treating acute ischemic stroke (AIS) patients with Emergent Large Vessel Occlusion (ELVO). There are numerous tools that predict outcomes of ischemic stroke patients including those given IV tPA, however, there are no published tools for predicting outcomes after ET. Methods: From January 2015-March 2017, 109 AIS patients received ET. Patients with unsuccessful ET (TICI 0-2a) or with incomplete 90 day follow-up data were excluded, leaving 42 for final analysis. Primary outcome was defined as MRS at 90 days (good outcome MRS <=3, bad outcome MRS >3). Of the variables analyzed, 90 day outcomes correlated with age, diabetes, thrombolytic use, onset to groin time, and NIHSS scores. Numerical values were assigned to each variable based on OR analysis and the resulting score (range 0-8) was plotted against 90 day MRS and ROC analysis defined a cutoff value. Results: The relative score for each non-binary variable was approximated based on the corresponding OR identified during ROC analysis. Using the DamAGE cONTrol scoring tool (figure 1), a cut off of 4 points yielded Sn 0.82, Sp 0.95, p <0.001, & AUC 0.94 . Conclusion: DamAGE cONTrol represents a novel scoring tool which uses pre-intervention characteristics to predict outcome after successful ET. This type of tool may facilitate more informed discussions regarding the value of performing ET, as well as control unnecessary transfers.