Anaesthetic management of an abdominal aortic aneurysmorrhaphy in Klippel-Trenaunay-Weber syndrome: a case report

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Abstract

Background

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.

Case presentation

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.

Conclusions

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.

Related collections

Most cited references 8

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European Society for Vascular Surgery (ESVS) 2019 Clinical Practice Guidelines on the Management of Abdominal Aorto-iliac Artery Aneurysms

Anders Wanhainen, Fabio Verzini, Isabelle Van Herzeele … (2018)

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Klippel and Trénaunay's syndrome. 768 operated cases.

M Servelle (1985)

Since 1945, we have operated on 786 patients with Klippel and Trénaunay's syndrome. Elongation of the impaired limb was invariably found while edema was present in 84%, varicose veins in 36%, and flat angiomata in 32%. Venography and surgical exploration have demonstrated malformation of the deep veins involving the popliteal vein in 51%; superficial femoral vein, 16%; both popliteal and superficial femoral veins; 29%; iliac veins, three per cent; and lower vena cava, one per cent. Good clinical results have been achieved following the surgical release of these deep veins in the lower limb. During childhood, when the difference in limb length is noteworthy, ligature of the popliteal vein of the shorter limb induces a compensating elongation. Klippel and Trénaunay's syndrome may be associated with lymphatic malformations, including lymphedema and malformation of the lymph vessels. Knowledge of the pathophysiology of these malformations of the deep veins enables a better understanding of the clinical manifestations of the condition, as well as the improved treatment of the serious vesical or rectal hemorrhage which occurs in one per cent of these patients.

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Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics.

David Barbara, Jack Wilson (2011)

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of varicose veins or venous malformations, capillary malformations that may involve neurovascular structures, and bony or soft tissue hypertrophy in affected limbs. Areas such as the trunk, bowel, bladder, and spinal cord may be involved as well. KTS should not be confused with Klippel-Feil syndrome, which involves abnormalities of the cervical vertebrae. Anesthetic management for patients with KTS has only been described in limited case reports that caution about potential airway difficulty but do not report surgical hemorrhage requiring transfusion.

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Author and article information

Contributors

Yuichi Tanaka:

ORCID: http://orcid.org/0000-0003-3085-0535

aolpril@nms.ac.jp

Journal

Journal ID (nlm-ta): BMC Anesthesiol

Journal ID (iso-abbrev): BMC Anesthesiol

Title: BMC Anesthesiology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2253

Publication date (Electronic): 11 July 2022

Publication date PMC-release: 11 July 2022

Publication date Collection: 2022

Volume: 22

Electronic Location Identifier: 214

Affiliations

[1 ]GRID grid.410821.e, ISNI 0000 0001 2173 8328, Department of Anesthesiology, , Musashikosugi Hospital, Nippon Medical School, ; 1-383, Kosugicho, Nakahara-ku, 211-8533 Kawasaki, Kanagawa Japan

[2 ]GRID grid.410821.e, ISNI 0000 0001 2173 8328, Department of Cardiovascular Surgery, , Musashikosugi Hospital, Nippon Medical School, ; 1-383, Kosugicho, Nakahara-ku, 211-8533 Kawasaki, Kanagawa Japan

[3 ]GRID grid.410821.e, ISNI 0000 0001 2173 8328, Department of Anesthesiology, , Nippon Medical School, ; 1-1-5, Sendagi, Bunkyo-ku, 113-8603 Tokyo, Japan

Author information

Yuichi Tanaka http://orcid.org/0000-0003-3085-0535

Article

Publisher ID: 1761

DOI: 10.1186/s12871-022-01761-y

PMC ID: 9275026

PubMed ID: 35820847

SO-VID: 1ee4a66e-59ba-4f47-a5d8-f23595c7d074

License:

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