Current Diagnostic and Therapeutic Approaches in May–Thurner Syndrome in Children, Adolescents, and Young Adults: A Survey among Thrombosis Experts of the German Society of Thrombosis and Haemostasis

Abstract

May–Thurner syndrome (MTS) is a pelvic venous disorder involving compression of the left common iliac vein by the right common iliac artery, which results in predisposition for deep vein thrombosis. Although MTS is increasingly recognized in young patients, specific guidelines on diagnosis and management for children, adolescents, and young adults do not exist so far. The aim of this study was to assess current diagnostic and therapeutic practice in Germany, Austria, and Switzerland in children and young adults with thrombosis and MTS.

We designed an online survey with 11 questions, which we sent via a mailing list to all members of the German, Austrian, and Swiss Society of Thrombosis and Haemostasis Research. Between July and October 2022, 33 specialists answered the questionnaire. Most participating specialists worked at pediatric hospitals (61%). Numbers of annually treated thromboses ranged from <5 (26%) to >30 (13%). Most specialists used venous ultrasound to diagnose deep vein thrombosis, 53% magnetic resonance imaging. Only 25% of specialists systematically screened for MTS in deep vein thrombosis. MTS was managed with anticoagulation (65%), iliac vein stent placement (32%), or balloon angioplasty (13%). In total, 31% of specialists reported to use more than one therapeutic method. Diagnostic and therapeutic approaches for MTS differed between specialists. Lack of standardization resulted in individualized and highly diverse management. Prospective observational clinical studies investigating the outcome of different management strategies including long-term follow-up on outcome and incidence of postthrombotic syndrome will help in defining patient groups who benefit most from revascularizing interventional strategies and developing standardized guidelines.

Zusammenfassung

Als May–Thurner Syndrom (MTS) wird eine Erkrankung der Beckenvenen bezeichnet, bei der die linke V. iliaca communis durch die rechte A. iliaca communis komprimiert wird, was zu einer Prädisposition für tiefe Bein-/ Beckenvenenthrombosen führt. Unter pädiatrischen Patienten sind vor allem Jugendliche und junge Erwachsene (AYA) betroffen. Spezifische Leitlinien zur optimalen Behandlungsstrategie für Kinder und AYA fehlen.

Ziel dieser Studie war, den derzeitigen diagnostischen und therapeutischen Standard für MTS bei diesen Patientinnen und Patienten in Deutschland, Österreich und der Schweiz zu erfassen.

Wir entwarfen eine Online-Umfrage mit 11 Fragen, die wir per Mailingliste an alle Mitglieder der Deutschen Gesellschaft für Thrombose- und Hämostaseforschung verschickten. Zwischen Juli und Oktober 2022 wurde der Fragebogen von 33 ExpertInnen beantwortet. Die meisten teilnehmenden Zentren waren Kinderkliniken (61%). Die Zahl der jährlich behandelten Thrombosen lag bei <5 (26%) bis >30 (13%). Die meisten ExpertInnen verwendeten den venösen Ultraschall zur Diagnose tiefer Venenthrombosen, 53% der ExpertInnen setzten auch die Magnetresonanztomographie ein. Nur 25% der ExpertInnen führten bei tiefen Venenthrombosen ein systematisches Screening auf MTS durch. Das MTS wurde mit Antikoagulation (65%), Stentimplantation in die V. iliaca (32%) oder Ballonangioplastie (13%) behandelt. 31% der ExpertInnen gaben an, mehr als eine Behandlungsmethode zu nutzen. Diagnostische und therapeutische Ansätze für das MTS unterschieden sich zwischen den ExpertInnen. Die fehlende Standardisierung führte zu einer individualisierten und unterschiedlichen Behandlung.

Prospektive Beobachtungsstudien, die die Ergebnisse verschiedener Behandlungsstrategien einschließlich der Inzidenz des postthrombotischen Syndroms in der Langzeitnachverfolgung untersuchen, werden dazu beitragen, Patientengruppen zu definieren, die von revaskularisierenden interventionellen Strategien profitieren, und standardisierte Leitlinien zu entwickeln.

Keywords

venous thrombosis - May–Thurner syndrome - iliac vein compression syndrome - children - adolescents - young adults

Schlüsselwörter

Thrombose - May–Thurner Syndrom - Vena-iliaca-Kompressionssyndrom - Kinder - Jugendliche - junge Erwachsene

Author Contributions

F.C., S.H., B.G., and A.G. designed the questionnaire. F.C. and S.H. collected data from the participating centers and wrote the manuscript. F.C. presented the results of the survey at the annual meeting of the German Society for Thrombosis and Haemostasis (GTH) 2023. E.L.-L. treats adult patients with MTS, has read the manuscript, and suggested modifications. All authors revised the manuscript.

Publication History

Received: 24 September 2023

Accepted: 06 March 2024

Article published online:
26 June 2024

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