Department of Anesthesiology, Musashikosugi Hospital, Nippon Medical School, 1-383, Kosugicho, Nakahara-ku, 211-8533, Kawasaki, Kanagawa, Japan.
Department of Cardiovascular Surgery, Musashikosugi Hospital, Nippon Medical School, 1-383, Kosugicho, Nakahara-ku, 211-8533, Kawasaki, Kanagawa, Japan.
BMC Anesthesiol. 2022 Jul 11;22(1):214. doi: 10.1186/s12871-022-01761-y.
Klippel-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.
A 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.
Regarding 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.
Klippel-Trenaunay-Weber 综合征（KTWS）是一种罕见的先天性畸形。尽管关于 KTWS 患者的麻醉管理已有少数报道，但这些患者的腹主动脉瘤（AAA）手术的麻醉管理数据却很少。
一名 74 岁男性（身高 160cm，体重 51.5kg）患有 KTWS，拟行 AAA 置换术。腹部 CT 显示腹主动脉下段明显迂曲，存在肾下型腹主动脉瘤、右髂总动脉瘤和右髂外动脉瘤。此外，在动脉瘤和外周动脉之间形成了一个明显的动静脉瘘。全身麻醉诱导后，在左侧颈内静脉插入了中心静脉导管和 8.5 法国鞘。在手术过程中，由于夹闭的主动脉中的侧支血管出血，外科医生决定进行动脉瘤缝合术。术中出血 1500ml，使用了 20 个单位的浓缩红细胞。
对于 KTWS 患者的 AAA 手术，由于侧支血管的存在，主动脉夹闭可能无法充分阻断血流。在这些患者中，麻醉师必须准备比正常的 AAA 手术更快速和频繁地输血。
