Shinban Liu, DO, Vadim Meytes, DO, Maria Roberto, DO. NYU Langone Hospital – Brooklyn
Introduction: Polyarteritis nodosa (PAN) is a systemic transmural inflammatory vasculitis that affects medium-sized arteries. Inflammation of the vessel wall and intimal proliferation creates luminal narrowing which can lead to stenosis and insufficiency. The same inflammatory process causes disruption of the elastic lamina leading to aneurysm formation and possible spontaneous rupture with life-threatening bleeding. Multifocal segments of stenosis and aneurysm formation are characteristically identified as a “rosary sign” or “beads on a string”. Unlike other vasculitides, PAN does not involve small arteries or veins, and is not associated with anti-neutrophil cytoplasmic antibodies. We present the case of a 66 year old female with a significant intra-abdominal bleed that was explored and repaired primarily. She was subsequently found on angiogram and postmortem pathology to have findings consistent with PAN.
Case Presentation: 66 year old female who presented to the emergency department with abdominal pain followed by hemorrhagic shock and found to have a ruptured left hepatic artery aneurysm during exploratory laparotomy. This aneurysm was suture ligated with a successful outcome. A mesenteric arteriogram was performed the following day and demonstrated lesions consistent with PAN including aneurysms of the left gastric branches, right and left hepatic arteries, and beaded appearance of the iliac artery. However, 2 days after hospital discharge she developed massive pulmonary embolism from which she did not recover. Postmortem examination confirmed rupture of the left hepatic artery aneurysm in addition to gross anatomical and histological findings consistent with PAN.
Discussion: Polyarteritis nodosa is a systemic inflammatory vasculitis that causes intimal proliferation and elastic lamina disruption. This multifocal disruption of the vessel results in aneurysm formation alternating with stenosis creating a characteristic “rosary sign” on imaging. Spontaneous rupture of these aneurysms is rare and almost always fatal due to life-threatening hemorrhage. With acutely ruptured aneurysms, prompt diagnosis, aggressive resuscitation, and hemostasis through transarterial embolization or surgery is paramount for patient survival. While acute rupture of an aneurysm as the result of PAN is exceedingly rare, it must be considered as a differential diagnosis in the setting of acute abdominal pain and hemodynamic instability. In a patient known to have a medical history of PAN and aneurysm formation, routine monitoring and disease progression should be followed.
Presented at the SAGES 2017 Annual Meeting in Houston, TX.
Abstract ID: 84832
Program Number: P173
Presentation Session: iPoster Session (Non CME)
Presentation Type: Poster