Tag Archives: prophylaxis

PUBLISHED: Pulmonary AVM Embolization

Pulmonary AVM Embolization
Jelena Ivanis1; Andrew Ding1; Dennis Barbon1; Fabian Laage-Gaupp, MD2; Jeffrey Pollak, MD2
1Frank H. Netter, MD School of Medicine at Quinnipiac University
2Yale School of Medicine

Pulmonary arteriovenous malformations (PAVMs) are rare fistulous connections between pulmonary arteries and veins that, as in this case, are commonly associated with hereditary hemorrhagic telangiectasia (HHT). Embolotherapy, the mainstay of treatment for PAVMs, is a procedure in which the feeding arteries of a malformation are endovascularly occluded under fluoroscopic guidance. Effective and well-tolerated, embolotherapy has been shown to decrease right-to-left shunting following treatment and decrease risks of paradoxical embolization and lung hemorrhage and to improve pulmonary gas exchange and lung function. Patients are selected for treatment according to clinical suspicion for the presence of a PAVM and feeding artery diameter. The occlusion of PAVMs with arteries that exceed 2–3 mm in diameter is recommended.

Diagnostic contrast-enhanced pulmonary angiography is performed via injection of contrast through a percutaneous catheter to characterize and confirm PAVMs suitable for embolization. Lesions are then treated by catheter-directed placement of embolic material— vascular plugs in this case—into the feeding artery, terminating blood flow to the area of the lesion. Although multiple PAVMs may be embolized during a single session, in patients with HHT, who may present with large numbers of PAVMs, treatment is limited by maximum contrast dosage, and additional sessions may be performed if PAVMs remain perfused.

PUBLISHED: Prophylactic Total Gastrectomy for CDH1 Gene Mutation

Prophylactic Total Gastrectomy for CDH1 Gene Mutation
Zhi Ven Fong, MD, MPH; John T. Mullen, MD
Massachusetts General Hospital

Hereditary Diffuse Gastric Cancer (HDGC) syndrome is due to a mutation in the CDH1 gene that predisposes patients to a high lifetime risk of developing gastric cancer. As such, a total gastrectomy is typically recommended for patients with this syndrome. In this case, the patient presented with an incidentally discovered CDH1 mutation on genetic testing obtained after she was diagnosed with early-onset rectal cancer. In this video, Dr. Mullen at MGH demonstrates his technique for performing an open prophylactic total gastrectomy with a Roux-en-Y esophagojejunostomy reconstruction.