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

Franziska Cuntz; Bernhard Gebauer; Andreas Greiner; Nikola Hagedorn; Madlen Reschke; Wolfgang Eberl; Barbara Zieger; Edelgard Lindhoff-Last; Susanne Holzhauer

doi:10.1055/a-2282-4565

Hämostaseologie, Table of Contents

Hamostaseologie
DOI: 10.1055/a-2282-4565

Original Article

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

Franziska Cuntz

¹Department of Paediatric Oncology and Haematology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

,

Bernhard Gebauer

²Department of Radiology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

,

Andreas Greiner

³Clinic of Vascular Surgery, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin, Germany

,

Nikola Hagedorn

¹Department of Paediatric Oncology and Haematology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

,

Madlen Reschke

¹Department of Paediatric Oncology and Haematology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

,

Wolfgang Eberl

⁴Department of Paediatrics, Städtisches Klinikum Braunschweig, Braunschweig, Germany

,

Barbara Zieger

⁵Division of Paediatric Haematology and Oncology, Department of Paediatrics and Adolescent Medicine, Medical Centre, Faculty of Medicine, University of Freiburg, Freiburg, Germany

,

Edelgard Lindhoff-Last

⁶Cardiology Angiology Centre Bethanien, CCB Vascular Centre, CCB Coagulation Centre, Frankfurt, Germany

,

Susanne Holzhauer

¹Department of Paediatric Oncology and Haematology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

› Author Affiliations

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

Full Text

References

References
1 Meissner MH, Khilnani NM, Labropoulos N. et al. The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: a report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders. J Vasc Surg Venous Lymphat Disord 2021; 9 (03) 568-584
2 May R, Thurner J. The cause of the predominantly sinistral occurrence of thrombosis of the pelvic veins. Angiology 1957; 8 (05) 419-427
3 Eklof B, Perrin M, Delis KT, Rutherford RB, Gloviczki P. American Venous Forum, European Venous Forum, International Union of Phlebology, American College of Phlebology, International Union of Angiology. Updated terminology of chronic venous disorders: the VEIN-TERM transatlantic interdisciplinary consensus document. J Vasc Surg 2009; 49 (02) 498-501
4 Cockett FB, Thomas ML. The iliac compression syndrome. Br J Surg 1965; 52 (10) 816-821
5 Gagne PJ, Tahara RW, Fastabend CP. et al. Venography versus intravascular ultrasound for diagnosing and treating iliofemoral vein obstruction. J Vasc Surg Venous Lymphat Disord 2017; 5 (05) 678-687
6 Cohen CT, Kirk S, Desai SB, Kukreja KU, Srivaths L. Diagnosis, clinical characteristics, and treatment modalities of adolescent May-Thurner syndrome-associated deep venous thrombosis. J Pediatr Hematol Oncol 2021; 43 (03) e346-e350
7 Zucker EJ, Ganguli S, Ghoshhajra BB, Gupta R, Prabhakar AM. Imaging of venous compression syndromes. Cardiovasc Diagn Ther 2016; 6 (06) 519-532
8 White JM, Comerota AJ. Venous compression syndromes. Vasc Endovascular Surg 2017; 51 (03) 155-168
9 Avila L, Cullinan N, White M. et al. Pediatric May-Thurner Syndrome-Systematic review and individual patient data meta-analysis. J Thromb Haemost 2021; 19 (05) 1283-1293
10 Poyyamoli S, Mehta P, Cherian M. et al. May-Thurner syndrome. Cardiovasc Diagn Ther 2021; 11 (05) 1104-1111
11 Miglioretti DL, Johnson E, Williams A. et al. The use of computed tomography in pediatrics and the associated radiation exposure and estimated cancer risk. JAMA Pediatr 2013; 167 (08) 700-707
12 Pearce MS, Salotti JA, Little MP. et al. Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet 2012; 380 (9840) 499-505
13 Ibrahim W, Al Safran Z, Hasan H, Zeid WA. Endovascular management of may-thurner syndrome. Ann Vasc Dis 2012; 5 (02) 217-221
14 Glonnegger H, Zieger B, Grohmann J. et al. Successful secondary endovascular intervention in pediatric patients with venous thromboembolic events. Hamostaseologie 2023; 43 (02) 102-109
15 Nawasrah J, Zydek B, Lucks J. et al. Incidence and severity of postthrombotic syndrome after iliofemoral thrombosis - results of the Iliaca-PTS - Registry. Vasa 2021; 50 (01) 30-37
16 Notten P, Ten Cate-Hoek AJ, Arnoldussen CWKP. et al. Ultrasound-accelerated catheter-directed thrombolysis versus anticoagulation for the prevention of post-thrombotic syndrome (CAVA): a single-blind, multicentre, randomised trial. Lancet Haematol 2020; 7 (01) e40-e49
17 Vedantham S, Goldhaber SZ, Julian JA. et al; ATTRACT Trial Investigators. Pharmacomechanical catheter-directed thrombolysis for deep-vein thrombosis. N Engl J Med 2017; 377 (23) 2240-2252
18 Comerota AJ, Kearon C, Gu CS. et al; ATTRACT Trial Investigators. Endovascular thrombus removal for acute iliofemoral deep vein thrombosis. Circulation 2019; 139 (09) 1162-1173
19 Notten P, de Smet AAEA, Tick LW. et al. CAVA (Ultrasound-Accelerated Catheter-Directed Thrombolysis on Preventing Post-Thrombotic Syndrome) trial: long-term follow-up results. J Am Heart Assoc 2021; 10 (11) e018973
20 Enden T, Haig Y, Kløw NE. et al; CaVenT Study Group. Long-term outcome after additional catheter-directed thrombolysis versus standard treatment for acute iliofemoral deep vein thrombosis (the CaVenT study): a randomised controlled trial. Lancet 2012; 379 (9810) 31-38
21 Betensky M, Goldenberg NA. Post-thrombotic syndrome in children. Thromb Res 2018; 164: 129-135
22 Avila L, Amiri N, De R. et al. Characteristics of upper- and lower-extremity deep vein thrombosis and predictors of postthrombotic syndrome in children. Blood Adv 2021; 5 (19) 3737-3747
23 Goldenberg NA, Branchford B, Wang M, Ray Jr C, Durham JD, Manco-Johnson MJ. Percutaneous mechanical and pharmacomechanical thrombolysis for occlusive deep vein thrombosis of the proximal limb in adolescent subjects: findings from an institution-based prospective inception cohort study of pediatric venous thromboembolism. J Vasc Interv Radiol 2011; 22 (02) 121-132
24 Bertino FJ, Hawkins CM, Woods GM. et al. Technical feasibility and clinical efficacy of iliac vein stent placement in adolescents and young adults with May-Thurner syndrome. Cardiovasc Intervent Radiol 2024; 47 (01) 45-59
25 Gefäßmedizin DGfAGf. Diagnostik und Therapie der Venenthrombose und Lungenembolie 2023. [Version 5.0: National AWMF guideline]. Accessed March 27, 2024 at: https://register.awmf.org/assets/guidelines/065-002l_S2k_Venenthrombose-Lungenembolie_2023-03.pdf
26 Ortel TL, Neumann I, Ageno W. et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv 2020; 4 (19) 4693-4738

Supplementary Material

Supplementary Material