Faculty Bibliography

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.

Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Although genetic confirmation is necessary for a definitive diagnosis, the radiologist serves as a central figure in the identification and treatment of these disorders. The clinical presentations, diagnostic and imaging workups, and treatment options available for patients with Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular anomalies, epidermal nevi, and scoliosis/spinal deformities) syndrome, fibroadipose vascular anomaly, phosphatase and tensin homolog mutation spectrum, Parkes-Weber syndrome, and Proteus syndrome are reviewed. ^©RSNA, 2019.