Lo CY, Chen KB, Chen LK, Chiou CS. Massive pulmonary embolism in Klippel-Trenaunay syndrome after leg raising: A case report. World J Clin Cases 2023; 11(17): 4133-4141 [PMID: 37388793 DOI: 10.12998/wjcc.v11.i17.4133]
Corresponding Author of This Article
Chiuan-Shiou Chiou, MD, PhD, Doctor, Department of Anesthesiology, China Medical University Hospital, No. 2 Yude Road, Taichung 40447, Taiwan. chiou.cs@gmail.com
Research Domain of This Article
Anesthesiology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Chih-Yu Lo, Li-Kuei Chen, Chiuan-Shiou Chiou, Department of Anesthesiology, China Medical University Hospital, Taichung 40447, Taiwan
Kuen-Bao Chen, Department of Anesthesiology Pain Service and Critical Care Medicine, China Medical University Hospital, Taichung 40447, Taiwan
Author contributions: Lo CY and Chen KB are the first authors; Lo CY and Chen KB conceptualized and drafted the initial manuscript, and reviewed and revised the manuscript; Chiou CS and Chen LK conceptualized and coordinated, reviewed, and revised the manuscript; All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chiuan-Shiou Chiou, MD, PhD, Doctor, Department of Anesthesiology, China Medical University Hospital, No. 2 Yude Road, Taichung 40447, Taiwan. chiou.cs@gmail.com
Received: March 12, 2023 Peer-review started: March 12, 2023 First decision: April 20, 2023 Revised: April 27, 2023 Accepted: May 12, 2023 Article in press: May 12, 2023 Published online: June 16, 2023 Processing time: 91 Days and 17.1 Hours
Abstract
BACKGROUND
Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by a combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. The syndrome predisposes patients to hypercoagulable states, including venous thromboembolism and pulmonary embolism (PE).
CASE SUMMARY
A 12-year-old girl with KTS was scheduled excision of verrucous hyperkeratosis in the left foot and posterior aspect of the left leg and left thigh and excision of a cutaneous hemangioma in the right buttock. After induction, the surgeon elevated the patient’s leg for sterilization, whereupon she experienced a massive PE and refractory cardiac arrest. Extracorporeal membrane oxygenation (ECMO) was performed after prolonged resuscitation, and she had a return of spontaneous circulation. After this episode, the patient was discharged without any neurologic complications.
CONCLUSION
The mechanism of PE, a lethal disease, involves a preexisting deep vein thrombosis that is mechanically dislodged by compression or changing positions and travels to the pulmonary artery. Therefore, patients predisposed to PE should be prescribed prophylactic anticoagulants. If the patient has unstable vital signs, resuscitation should be started immediately, and extracorporeal cardiopulmonary resuscitation should be considered in settings with existing ECMO protocols, expertise, and equipment. Awareness of PE in patients with KTS while leg raising for sterilization is critical.
Core Tip: Klippel-Trenaunay syndrome (KTS) is a congenital vascular disorder. Patients with KTS are at high risk for pulmonary embolism. All patient having KTS should be evaluated of lower limb circulation and pre-existing deep vein thrombosis (DVT) pre-operatively. Also, all patients with KTS should receive DVT prophylaxis at least 8 hours prior to surgery irrespective of the age. Care should be taken to monitor for PE in patients with KTS while leg raising for sterilization. Anesthesiologists should consider potential difficulties in managing the airway and avoid neuraxial anesthesia.
