Embolization of De Novo Pulmonary Arteriovenous Malformations Using High-Volume Detachable Non-Fibered Coils: Propensity-Matched Comparison to Traditional Coils.

Embolization of de novo pulmonary arteriovenous malformations (PAVMs) using high-volume detachable non-fibered (HVDNF) coils was compared to traditional non-HVDNF coils. Persistent-occlusion rates were evaluated. A total of 272 de novo (previously untreated) PAVM treatments were retrospectively stratified into those treated with non-HVDNF coils only (n = 192) and those treated with HVDNF coils with or without other coils (n = 80). Propensity score matching, followed by survival analysis and cost analysis, was performed. The overall persistent-occlusion rate was 86.0% (234/272). Persistent occlusion was achieved in 81.8% of PAVMs using non-HVDNF coils, compared with 96.3% using HVDNF coils (p = 0.0017). The mean follow-up was 30.7 ± 31.9 months versus 14.7 ± 13.4 months, respectively (p < 0.0001). Propensity-matched survival analysis demonstrated PAVMs treated with HVDNF coils recurred significantly less frequently than PAVMs treated with non-HVNDF coils (p = 0.023). The use of HVDNF coils was more expensive than standard coils, however not significantly different for the treatment of complex PAVMs. The use of high-volume detachable non-fibered coils was associated with higher persistent-occlusion rates when compared with non-HVDNF coils. HVDNF coils were more expensive on average; however, cost was similar between groups for the treatment of complex PAVMs.

Biotin-thiamine-responsive basal ganglia disease: A case report.

Biotin-Thiamine-Responsive Basal Ganglia Disease is an extremely rare autosomal recessive neurometabolic disorder characterized by recurrent waxing and waning episodes of subacute encephalopathy and seizures. High dose biotin and thiamine administration has been shown to improve symptoms within days, and the symptoms may reappear rapidly if supplementation is discontinued. Here we present a case of a 20-year-old male with classical clinical and imaging findings of Biotin-Thiamine-Responsive Basal Ganglia Disease, with a 12-year delay in diagnosis, finally diagnosed after presenting at our institution based on imaging and subsequent reexamination of exome sequencing. In this report, we review the classic imaging findings in this disease and examine why making the diagnosis can be extremely challenging due to its wide differential. Both clinically and radiographically, this condition demonstrates significant overlap with a vast array of disease entities, ranging from viral or autoimmune encephalitis to metabolic disorders. Finally, we discuss the various negative prognostic predictors described in the literature, several of which were observed in this patient's clinical course.

North American Study for the Treatment of Recurrent Epistaxis with Doxycycline: The NOSTRIL trial.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a vascular bleeding disorder characterized by mucocutaneous telangiectasias and visceral arteriovenous malformations. A frequently debilitating symptom is spontaneous recurrent epistaxis. OBJECTIVE: To evaluate whether doxycycline therapy improves epistaxis in HHT by using a prospective, randomized, placebo-controlled crossover study design. PATIENTS/METHODS: Twenty-two eligible patients between December 2017 and July 2020 at a single center were randomized to one of two study arms: doxycycline treatment followed by placebo, or vice versa. Primary outcomes measured differences in epistaxis severity between treatments. Changes in quality of life, laboratory markers of bleeding, and number of monthly blood transfusions or iron infusions were assessed as secondary endpoints. Additional post hoc endpoints included frequency and duration of dripping epistaxis and gushing epistaxis. A post hoc longitudinal analysis assessed effects of doxycycline over time. RESULTS/CONCLUSIONS: Doxycycline was safe and well tolerated. However, there was no reduction in the three primary outcome measures, nosebleed frequency (p = .16), nosebleed duration (p = .05), and Epistaxis Severity Score (p = .19). Quality of life, hemoglobin level, and number of blood transfusions and iron infusions did not differ between groups. Post hoc analysis demonstrated reduction in instances of gushing (p = .02) with doxycycline, although this finding is of unclear clinical significance. Post hoc longitudinal analysis showed reduction in frequency (mean estimate of coefficient = -0.19, standard error = 0.07, p = .01) and duration (mean estimate of coefficient = -2.33, standard error = 1.08, p = .03) of epistaxis over time. Post hoc findings suggest possible benefit of doxycycline but should be interpreted with caution given the overall negative study. Further investigation is needed with a larger sample size and a longer treatment duration.

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update.

Pulmonary arteriovenous malformations (PAVMs) are abnormal direct vascular communications between pulmonary arteries and veins which create high-flow right-to-left shunts. They are most frequently congenital, usually in the setting of hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with a wide variety of clinical manifestations such as dyspnea, hypoxemia, or chest pain. Even when asymptomatic, presence of PAVMs increases patients' risk of serious, potentially preventable complications including stroke or brain abscess. Transcatheter embolotherapy is considered the gold standard for treatment of PAVMs. Though previous guidelines have been published regarding the management of PAVMs, several aspects of PAVM screening and management remain debated among the experts, suggesting the need for thorough reexamination of the current literature. The authors of this review present an updated approach to the diagnostic workup and management of PAVMs, with an emphasis on areas of controversy, based on the latest literature and our institutional experience.

Embolization of a congenital arteriovenous malformation arising off the internal mammary artery.

Congenital arteriovenous malformations (AVM) of the internal mammary artery (IMA) are exceptionally rare. Patients with AVMs arising off the left IMA may present with continuous precordial murmurs and/or a pulsatile chest mass. AVMs in this location pose a risk of enlargement, rupture, infection, or high-output cardiac failure. The potential risks associated with AVMs warrant early therapeutic intervention. We describe a case of a congenital AVM originating from the left IMA, which was successfully embolized with n­butyl cyanoacrylate (NBCA) using a transcatheter arterial approach.

Endovascular Management of SVC Syndrome due to Fibrosing Mediastinitis-A Feasibility and Safety Analysis.

PURPOSE: To assess the outcomes of endovascular management for superior vena cava (SVC) syndrome secondary to fibrosing mediastinitis (FM). METHODS: Between January 2004 and December 2016, 10 consecutive patients with endovascularly managed SVC syndrome secondary to FM were identified in an institutional database. Venograms were performed to assess the severity and location of the lesion and allow measurement for stent selection. Standard stenting and angioplasty techniques were utilized to establish luminal patency. The safety, feasibility, clinical success, and the primary and secondary patency were evaluated. Kaplan-Meier survival analysis was used to determine median duration of stent patency. A log-rank test was used to test differences in prior stent use. RESULTS: Our cohort was predominantly female (7/10) with an average age of 42.2 years. Of the 10 patients, 3 had undergone endovascular stenting at an outside institution prior to referral to our institution due to new, recurrent, or worsening symptoms. All patients underwent venography demonstrating stenosis (8/10) or occlusion (2/10) at initial presentation. Stenting or angioplasty was technically successful in 9 (90.0%) patients. Eight of 10 patients had primary stenting, while 1 achieved vascular patency and symptom resolution with angioplasty alone. Median duration of primary patency was 31.3 months (95% confidence interval: 5.9-103). Six (54.5%) patients required secondary revision procedures. Median duration of secondary patency was 6.1 months, with 25% of revisions occurring by 4 months and 75% occurring by 20.9 months. All treated patients (9/9) reported symptomatic relief at 1-month follow-up, establishing a clinical success rate of 100%. There were no 30-day adverse effects related to the procedure. CONCLUSION: This study demonstrates that endovascular therapy is a safe and feasible approach for managing FM-related SVC syndrome.