Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa

Abstract

Polyarteritis nodosa (PAN) is an inflammatory vasculitis that creates regions of stenosis and aneurysm formation. The authors describe a 66-year-old female with hepatic artery rupture as the first presentation of undiagnosed PAN, presenting with abdominal pain followed by hemorrhagic shock. This aneurysm was suture ligated with a successful outcome. A mesenteric arteriogram 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, she developed massive pulmonary embolism from which she did not recover after discharge. Postmortem examination confirmed left hepatic artery aneurysm rupture and changes consistent with PAN on gross anatomical examination and histology. This report provides a unique overview of the disease process through imaging, gross anatomic specimen and pathology. Life-threatening hepatic artery aneurysm rupture is an uncommon presentation of PAN which may benefit readers in creating a more robust differential diagnosis.

Figure 1:

(a) A contrast-enhanced CT of the abdomen shows a large fluid collection in the region of the left upper quadrant, abutting the lesser curvature of the stomach. Questionable active extravasation of contrast medium (a, white arrow) in the region of the left hepatic artery with shoddy celiac axis noted (c). The CT numbers range from 130 to 200 Hounsfield units in the area, consistent with possible contrast material.

Figure 2:

(a and b) Above: Mesenteric angiography showing aberrant anatomy as well as numerous visceral aneurysms including the right and left hepatic, and left gastric branches, without active contrast extravasation. (c) A contrasted angiogram examination of the right external iliac and femoral arteries showing a beaded appearance of the iliac and normal femoral artery.

Figure 3:

(a–d) Above: Near saddle pulmonary embolism (a, yellow arrow). Aberrant arterial anatomy with hepatic artery originating from the SMA. Left hepatic artery with suture ligation and thrombosis (b). Left hepatic artery aneurysm (b, white circle). Full thickness inflammatory necrosis with a pseudoaneurysm that had dissected (c). Full thickness inflammatory necrosis of the left hepatic artery consistent with polyarteritis nodosa (d).