• Yuichi Tanaka, Shun-Ichiro Sakamoto, Hiroyasu Bito, and Atsuhiro Sakamoto.
• Department of Anesthesiology, Musashikosugi Hospital, Nippon Medical School, 1-383, Kosugicho, Nakahara-ku, 211-8533, Kawasaki, Kanagawa, Japan. aolpril@nms.ac.jp.
• BMC Anesthesiol. 2022 Jul 11; 22 (1): 214.

BackgroundKlippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital malformation. Although there have been few reports on anaesthetic management of patients with KTWS, there is a lack of data on anaesthetic management for abdominal aortic aneurysm (AAA) surgeries in these patients.Case PresentationA 74-year-old man (height, 160 cm and body weight, 51.5 kg) with KTWS was scheduled for AAA replacement. Abdominal computed tomography (CT) showed prominent tortuosity below the abdominal aorta with an infrarenal abdominal aortic aneurysm, right common iliac artery aneurysm, and right external iliac artery aneurysm. Moreover, a remarkably noted arteriovenous fistula had developed between the aneurysm and peripheral artery. General anaesthesia was induced. Furthermore, a central venous catheter and an 8.5 French sheath in the left internal jugular vein were inserted. During the operation, bleeding from a collateral vessel in the cross-clamped aorta led the surgeon to decide to perform aneurysmorrhaphy. Intraoperatively, blood loss was 1500 ml, and 20 units of red blood cell concentrate were used.ConclusionsRegarding AAA procedures in patients with KTWS, aortic cross-clamping may not sufficiently intercept blood flow due to collateral vessels. In these patients, the anaesthesiologist must be prepared to transfuse blood more rapidly and frequently than during normal AAA procedures.© 2022. The Author(s).

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