Faculty Bibliography

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.

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.

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.

Petrus Camper's contributions to modern anatomical science include descriptions of the foot, upper limb, axilla, and inguinal region. His explanation of the etiology of inguinal hernias revolutionized the surgical practice of their repair. Camper's description of abdominal anatomy was an invaluable contribution to the field of abdominal surgery. Current research reviewed in this article shows the importance of understanding the layers of the abdominal wall, notably Camper's fascia, the closure of which has been found to be vital to proper wound healing and patient recovery. This article begins with a biography of Petrus Camper and his research in the inguinal region. It continues with anatomical and histological descriptions of Camper's fascia and finishes with its clinical correlates in surgical practice.