Nonatherosclerotic Abdominal Vasculopathies.

BACKGROUND: Nonatherosclerotic abdominal arterial vasculopathies (NAVs), including mesenteric or renal artery dissection, aneurysm, stenosis, and vasculitis, are rare but have great clinical significance. Patients may present emergently with life-threatening complications such as arterial rupture and hemorrhagic shock. Herein, we present our center's experience with NAVs and provide extensive literature review to close the gap in the scarce, related literature. METHODS: From a single-center retrospective data analysis, we identified and characterized subjects (aged 18-60 years) who presented with NAV between January 2000 and December 2015. Of the 1416 charts reviewed, 118 met inclusion criteria. RESULTS: The average age of patients with NAV was 47.0 ± 9.9 years, mostly affecting women (64%). Primary diagnoses included fibromuscular dysplasia (FMD) (25.4%), isolated aneurysms (24.6%), and median arcuate ligament syndrome (MALS) (15.3%). Less common diagnoses were localized vasculitis of the gastrointestinal tract (LVGT) (7.6%), isolated dissection (5.1%), microscopic polyangiitis and granulomatosis with polyangiitis (5.1%), trauma (4.2%), segmental arterial mediolysis (4.2%), Ehlers-Danlos syndrome (2.5%), Takayasu's arteritis (2.5%), polyarteritis nodosa (1.7%), idiopathic abdominal aortitis (0.8%), and Loeys-Dietz syndrome (0.8%). Females constituted 90% of patients with FMD, 77.8% with MALS, 77.8% with isolated aneurysms, 66.7% with Takayasu arteritis, and 55.6% with LVGT. Prevalent comorbidities included tobacco use (43.6%) and hypertension (52.1%). Coil embolization was used in 14.4%, anticoagulation in 11.9%, angioplasty/stenting in 11.9%, open resection/surgical revascularization in 10.2%, and prednisone in 10.2% of the cases. Conservative management was pursued in 33.1% of the patients. A high degree of symptom relief was shown in 91.7%. CONCLUSIONS: NAV are rare and can be caused by different etiologies that primarily affect females. Hypertension and tobacco use were prevalent. Various imaging strategies revealed aneurysms, stenosis, dissection, and/or thrombosis affecting renal and celiac arteries. Most patients improved with conservative, medical, endovascular, or surgical approach. More research is needed to standardize management approach to patients with NAV.

Medical Management of Extensive Arterial Thromboembolism in a Patient with Essential Thrombocythemia and Warfarin Failure.

Chronic myeloproliferative disorders share a stem cell-derived clonal myeloproliferation. This group of disorders include essential thrombocythemia (ET), polycythemia vera (PV), chronic myeloid leukemia, and primary myelofibrosis (PMF), with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis. These disorders can be associated with genetic mutations affecting protein tyrosine kinases, resulting in different configurations of abnormal signal transduction. The Janus tyrosine kinase 2 mutation can be used as a key diagnostic tool for diagnosing MPDs, specifically, ET, PV, and PMF. Patients with ET and PV are at an increased risk for thromboembolic and hemorrhagic events. We present a unique case of ET causing extensive arterial thromboembolism, despite being on adequate antithrombotic agents including warfarin and aspirin.

Large thoracic aortic aneurysm and dissection with rare complication.

Thoracic aortic aneurysms can be found incidentally, however, patients can also present with acute dissection and or rupture that can be fatal. Symptoms that might indicate dissection include chest and back pain as well as lightheadedness. The diagnosis can be made with imaging studies such as computed tomography or magnetic resonance angiogram and sometimes transesophageal echocardiogram. Management is based on the aneurysmal size, location, extension, and the presence of complications. Although smaller localized and slow growing aneurysms can be monitored, larger and or complicated ones may warrant immediate repair. Less-common complications include compression over anatomic structures in the vicinity including vessels and the mediastinum. We report a unique case of a 71-year-old man who presented with a very large thoracic aortic aneurysm with dissection causing compression over the brachiocephalic veins and the mediastinum leading to facial and upper extremity swelling, dysphagia, and cough. This case represents a rare but significant complication of thoracic aortic aneurysm and emphasizes the challenges of its management.