Pancreaticoduodenal artery aneurysms and cerebral aneurysms in a patient of Sjögren’s syndrome: a surgical case report and literature review

Pancreaticoduodenal artery aneurysms (PDAAs) are associated with celiac axis stenosis and occlusion. PDAAs carry a risk of sudden rupture and therefore require prompt treatment. Sjögren’s syndrome (SS), a systemic autoimmune disease, has recently been reported in association with various arterial aneurysms. To our knowledge, no previous report has described concurrent PDAAs and cerebral aneurysms (CAs) associated with SS. Here, we present a rare case of PDAAs and CAs associated with SS. A 69-year-old Japanese woman with a history of chronic hepatitis was referred to our hospital. She had a 20-year history of dry mouth, a one-month history of taste disturbance, and joint pain. Laboratory tests revealed she was SS. Abdominal ultrasonography performed for chronic hepatitis incidentally revealed aneurysms around the pancreas. Contrast-enhanced computed tomography (CECT) demonstrated multiple PDAAs, including two anterior superior PDAAs, three anterior inferior PDAAs, one inferior PDAA, and two dorsal pancreatic artery aneurysms. The origin of the celiac axis was occluded. CECT also revealed three CAs: an anterior communicating artery (AcomA) aneurysm with a bleb, a right middle cerebral artery aneurysm, and a right M1 bifurcation aneurysm. We planned a three-stage treatment strategy. First, an aorto-hepatic bypass using a reversed great saphenous vein graft was performed. The graft was proximally anastomosed to the abdominal aorta 15 mm above the inferior mesenteric artery, routed through the mesocolon near the ligament of Treitz, and passed between the stomach and pancreas. It was then distally anastomosed to the common hepatic artery. The gastroduodenal artery (GDA) was ligated to prevent competitive flow between the GDA and the graft. The postoperative course was uneventful, and the patient was discharged on postoperative day 9. Twenty days later, coil embolization of the PDAAs was performed as the second treatment. Fifty days thereafter, stent-assisted coil embolization of the AcomA aneurysm was performed as the third treatment. Twelve months after the aorto-hepatic bypass, the graft remained patent, and the sizes of the remaining CAs were unchanged. Our case highlights the potential for systemic aneurysm formation in patients with SS and demonstrates that staged multidisciplinary treatment can be safely and effectively performed.