title: Multimodal endovascular management of a Jehovah's Witness patient with Hugues-Stovin syndrome presenting with ruptured pulmonary artery aneurysm and cardiopulmonary thromboembolism.
abstract: Hughes-Stovin syndrome is a very rare disease with fewer than 40 cases reported in the literature. The disease is thought to be a variant of Behçet's disease that can manifest with massive haemoptysis due to ruptured pulmonary arterial aneurysms in the setting of deep venous thrombosis. This association might impose a therapeutic dilemma when anticoagulation has to be balanced with the excessive risk of bleeding, especially if the patient refuses any blood replacement, and is not a surgical candidate. We present a case of a 41-year-old Jehovah's Witness patient with Hughes-Stovin syndrome that presented to the emergency room with haemoptysis due to multiple ruptured pulmonary artery aneurysms. Her work-up showed concomitant cardio-venous thromboembolism and pulmonary infarction. She was successfully managed via a bloodless single-wall venous puncture, allowing endovascular treatment of bilateral pulmonary aneurysms with subsequent caval filtration. The course was uneventful, and the patient was discharged under immunosuppressant.
authors: Department of Radiology, Section of Vascular and Interventional Radiology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, Miami, FL, USA ikably@med.miami.edu.
authors: Issam M
authors: IM
authors: Kably
authors: Department of Radiology, University of Miami Miller School of Medicine, Miami, FL, USA.
authors: Carolina
authors: C
authors: Reveron
mesh_terms: D000328:Adult; D017542:Aneurysm, Ruptured / Q000601:surgery*; D001528:Behcet Syndrome / Q000150:complications*; D057510:Endovascular Procedures / Q000379:methods*; D005260:Female; D006801:Humans; D033221:Jehovah's Witnesses; D011651:Pulmonary Artery / Q000601:surgery*; D011655:Pulmonary Embolism / Q000601:surgery*
keywords: Aneurysm; Embolization; Endovascular; Hughes–Stovin syndrome