Faculty Bibliography

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.

Research and other scholarly activities are an important and required component of diagnostic radiology training. Several strategies, both at the departmental and the larger organizational levels, have been implemented to encourage radiology trainees to participate in these activities. In this article, we review and discuss our institution's 10-year experience in supporting the development and realization of scholarly projects through a competitive intramural grant for residents.

PURPOSE:To examine the technical feasibility and clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in children and adolescents. MATERIALS AND METHODS:Retrospective review was performed of 59 patients (mean age 12.6 y [range, 1.5-20 y], mean weight 47.5 kg [range, 11.4-112.2 kg], mean Model for End-stage Liver Disease/Pediatric End-stage Liver Disease score 12.5 [range, 6-33]) who underwent 61 TIPS attempts at 3 tertiary children's hospitals from 2001 to 2017 for acute esophageal or gastroesophageal variceal bleeding, primary and secondary prevention of variceal bleeding, and refractory ascites. Pediatric liver disease etiologies included biliary atresia, cystic fibrosis, and ductal plate anomalies. Technical, hemodynamic, and clinical success and patency rates were reported at 1, 6, 12, and 24 months. Statistical analysis evaluated reasons for clinical failure. Kaplan-Meier analysis measured clinical success, patency, and transplant-free survival. RESULTS:Technical success was 93.4% (57/61) in 59 consecutive patients. Most common TIPS indications were treating and preventing esophageal and gastroesophageal variceal bleeding (57/59; 96.6%). Hemodynamic success was 94% (47/50). Clinical success was 80.7% (45/56). Two-year clinical success for acute variceal bleeding and ascites was 94.1% and 100%, respectively. Overall patency at 1, 6, 12, and 24 months was 98.0%, 97.8%, 94.3%, and 91.3%. Two-year transplant-free survival was 88.8%. Overall and major complication rates were 21.2% (13/61) and 8.2% (5/61), with 3 mortalities. Gradient reduction < 12 mm Hg correlated with clinical success (P < .01). CONCLUSIONS:TIPS creation in pediatric patients is technically feasible and clinically efficacious for treatment and prevention of esophageal and gastroesophageal variceal hemorrhage. High 2-year clinical success, patency, and survival rates should encourage providers to consider portosystemic shunts as a bridge to liver transplantation.