Faculty Bibliography

PURPOSE/OBJECTIVE:To report technical and clinical outcomes of thoracic central vein reconstruction for superior vena cava (SVC) syndrome using kissing Viabahn VBX stent-grafts. MATERIALS AND METHODS/METHODS:All adult patients with SVC syndrome who underwent attempted bilateral brachiocephalic vein-to-SVC reconstruction using kissing VBX stent-grafts at an academic hospital between August 2019 and February 2021 were reviewed. Technical results, adverse events, imaging follow-up, and clinical outcomes were recorded. Patency over time was assessed with Kaplan-Meier analysis. RESULTS:Twenty-eight patients (16 women, 12 men; mean age, 52.0 years) constituted the study cohort. Seventeen (60.7%) had benign, and 11 (39.3%) had malignant etiologies. Presenting symptoms included neck swelling (n=17; 60.7%), bilateral upper extremity swelling (n=15; 53.6%), dyspnea (n=7; 25%), unilateral upper extremity swelling (n=4; 14.3%), and dysphagia (n=1; 3.6%). SVC reconstruction with VBX stent-grafts in kissing configuration was successfully completed in 27 of 28 patients (96.4%). Four major adverse events were noted in the benign etiology subgroup (23.5%), including intraprocedural hemopericardium (n=3) and delayed pneumothorax (n=1). Twenty-seven (96.4%) patients experienced resolution of the presenting symptoms. Mean clinical follow-up of living patients was 358.8±77.2 days (range, 78-645 days). Mean imaging follow-up of living patients was 272.6±91 days (range, 26-594 days). The primary, primary-assisted, and secondary patency rates at 12-months were 71.8%, 88.8%, and 100%, respectively. CONCLUSION/CONCLUSIONS:For management of SVC syndrome, thoracic central vein reconstruction with kissing VBX stent-grafts is feasible with high rate of symptom resolution and acceptable patency. However, this technique is not recommended in benign SVC syndrome due to a high risk of cardiac tamponade.

Venous malformations, the most common type of vascular malformation, are slow-flow lesions resulting from disorganized angiogenesis. The International Society for the Study of Vascular Anomalies (ISSVA) classification offers a categorization scheme for venous malformations based on their genetic landscapes and association with congenital overgrowth syndromes. Venous malformations present as congenital lesions and can have broad physiologic and psychosocial sequelae depending on their size, location, growth trajectory, and tissue involvement. Diagnostic evaluation is centered around clinical examination, imaging evaluation with ultrasound and time-resolved magnetic resonance imaging, and genetic testing for more complex malformations. Interventional radiology has emerged as first-line management of venous malformations through endovascular treatment with embolization, while surgery and targeted molecular therapies offer additional therapeutic options. In this review, an updated overview of the genetics and clinical presentation of venous malformations in conjunction with key aspects of diagnostic imaging and treatment are discussed.

External lumbar drain placement has been shown to be an efficacious and safe approach to managing various forms of intracranial hypertension in adult patients and children. The use of ultrasound guidance for lumbar punctures in young patients has been described however, but the modality is not routinely used for the placement of tunneled lumbar drains. In this report, two cases are presented that detail experience using ultrasound guidance for tunneled lumbar drains in children.

BACKGROUND:Portomesenteric and portosystemic venous occlusive disease may lead to portomesenteric hypertension, variceal bleeding, ascites and hypersplenism. Data regarding endovascular reconstructive strategies in children, however, are limited. OBJECTIVE:To report technical success, outcome and patency of portomesenteric and portosystemic venous reconstruction using VIABAHN VBX balloon-expandable endoprostheses in pediatric patients. MATERIALS AND METHODS/METHODS:Five pediatric patients (median age: 15 years, range: 4-18 years), including 3 (60%) boys and 2 (40%) girls, with portomesenteric or portosystemic venous occlusion or recurrent stenosis, underwent balloon-expandable stent graft reconstruction. Presenting symptoms included acute variceal bleeding, without (n = 2, 40%) or with (n = 1, 20%) splenomegaly, and transfusion-dependent chronic melena (n = 1, 20%). One patient was asymptomatic (n = 1, 20%). Preprocedural imaging included Doppler ultrasound and contrast-enhanced computed tomography (CT) in all patients. Initial imaging showed 4 (80%) occlusions and 1 (20%) recurrent stenosis greater than 50%. Technical aspects of the reconstructions, technical successes, clinical outcomes and adverse events were recorded. Technical success was defined as completion of stent graft reconstruction. Adverse events were categorized according to Society of Interventional Radiology criteria. Clinical success was defined as resolution of the presenting symptoms and/or prevention of portal hypertensive sequela. RESULTS:Venous reconstruction was technically successful in all five patients. Stent graft locations included the main portal vein in 2 (40%), the superior mesenteric vein in 1 (20%), autologous Meso-Rex shunt in 1 (20%) and splenocaval shunt in 1 (20%). Six stent grafts were placed (two stent grafts placed in a single patient). Stent grafts had a median diameter of 7 mm (range: 6-10 mm) and a median length of 59 mm (range: 19-79 mm). Median fluoroscopy time was 36.6 min (range: 13.4-95.8 min) and median air kerma was 301.0 mGy (range: 218.0-1,148.2 mGy). No adverse events occurred. Median clinical follow-up was 18 months (range: 6-29 months). Median imaging follow-up was 17 months (range: 2-29 months). Clinical success was achieved in all patients and maintained during the follow-up period. One patient required follow-up intervention with superior mesenteric vein side extension with a self-expanding bare metal stent due to perigraft stenosis detected on CT 3 months after stent placement. There were no stent graft occlusions. CONCLUSION/CONCLUSIONS:Portomesenteric and portosystemic venous reconstruction using balloon-expandable stent grafts in pediatric patients was feasible and clinically successful in this preliminary experience. Additional studies are warranted.

Vascular malformations and vascular tumors comprise the two specific subsets of vascular anomalies that arise as a result of disorganized angiogenesis and neoplasm, respectively. Malformations are separate entities from vascular tumors (e.g., hemangiomas) and are recognized by the International Society for the Study of Vascular Anomalies (ISSVA) as such. Vascular malformations are classified into four main groups: simple, combined, anomalies of major vessels, and those associated with other vascular anomalies. Vascular tumors are neoplastic growths of blood vessels and are morphologically and molecularly distinct from malformations but can arise in the head and neck and have syndromic association. Head and neck vascular anomalies are not uncommon in the pediatric population and require special care in the workup, diagnostic imaging and clinical care. The purpose of this manuscript is to discuss the diagnosis and management of the most common intracranial and extracranial vascular malformations and tumors in the head and neck in children and adolescents.