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DOI: 10.1055/s-0040-1714694
Clinical Outcomes of May–Thurner Syndrome in Pediatric Patients: A Single Institutional Experience
Abstract
Introduction May–Thurner syndrome (MTS) is a vascular anatomic variant resulting in compression of the left common iliac vein by the right common iliac artery, affecting approximately 22% of the population. In adults, following acute deep vein thrombosis (DVT) of the iliofemoral veins, the incidence of postthrombotic syndrome (PTS) and recurrent DVT are high if treated with anticoagulation alone, warranting adjunctive treatment with thrombolysis and stent placement. However, there is paucity of literature documenting the course of treatment and associated outcomes in pediatric patients with MTS.
Methods A retrospective chart review of pediatric patients (≤ 18 years of age) with radiologic confirmation of MTS with or without DVT evaluated and/or treated at our institution from January 1, 2005 through December 31, 2015 was conducted.
Results Seventeen patients (4 male; 13 female) were identified. Median age was 15.4 years (range 8.8–17.1 years) with a median follow-up of 1.2 years (range 0.4–7.5 years). Thirteen (76.5%) patients presented with left lower extremity DVT. Management included catheter-directed thrombolysis (n = 5), systemic thrombolysis (n = 1), and mechanical thrombectomy (n = 1). Fifteen patients were treated with anticoagulation including two patients with endovascular stents without DVT. Median duration of anticoagulation was 6.3 months (range 3.2–18.7 months). Ten patients (59%) underwent stent placements.
Complete and partial thrombus resolution was noted in six patients each and no resolution in one patient. Four patients had recurrence/progression of thrombus (n = 3 with stents) at a median time of 29 days (range 12–495 days). No bleeding complications were observed. Clinically documented or self-reported PTS was noted in 8 patients (62%).
Conclusion There are no clear guidelines for MTS management in children and adolescents. In our cohort, thrombolysis, anticoagulation, or stent placements were not associated with bleeding risks, with recurrence/progression of DVT and signs and symptoms of PTS noted in 30 and 62%, respectively. Further studies are needed to determine a standardized treatment approach of the pediatric patient with MTS with or without thrombosis.
Authors' Contributions
All authors have contributed significantly to this study and preparation of the manuscript. All authors have reviewed and approved the manuscript.
Publication History
Received: 30 April 2020
Accepted: 25 June 2020
Article published online:
20 August 2020
© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).
Georg Thieme Verlag KG
Stuttgart · New York
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References
- 1 May R, Thurner J. The cause of the predominantly sinistral occurrence of thrombosis of the pelvic veins. Angiology 1957; 8 (05) 419-427
- 2 Cockett FB, Thomas ML. The iliac compression syndrome. Br J Surg 1965; 52 (10) 816-821
- 3 Kibbe MR, Ujiki M, Goodwin AL, Eskandari M, Yao J, Matsumura J. Iliac vein compression in an asymptomatic patient population. J Vasc Surg 2004; 39 (05) 937-943
- 4 Mickley V, Schwagierek R, Rilinger N, Görich J, Sunder-Plassmann L. Left iliac venous thrombosis caused by venous spur: treatment with thrombectomy and stent implantation. J Vasc Surg 1998; 28 (03) 492-497
- 5 Rajachandran M, Schainfeld RM. Diagnosis and treatment of May-Thurner syndrome. Vasc Dis Manag 2014; 11 (11) E265-E273
- 6 Bozkaya H, Cinar C, Ertugay S. , et al. Endovascular treatment of iliac vein compression (May-Thurner) syndrome: angioplasty and stenting with or without manual aspiration thrombectomy and catheter-directed thrombolysis. Ann Vasc Dis 2015; 8 (01) 21-28
- 7 Mousa AY, AbuRahma AF. May-Thurner syndrome: update and review. Ann Vasc Surg 2013; 27 (07) 984-995
- 8 Hager ES, Yuo T, Tahara R. , et al. Outcomes of endovascular intervention for May-Thurner syndrome. J Vasc Surg Venous Lymphat Disord 2013; 1 (03) 270-275
- 9 Lamont JP, Pearl GJ, Patetsios P. , et al. Prospective evaluation of endoluminal venous stents in the treatment of the May-Thurner syndrome. Ann Vasc Surg 2002; 16 (01) 61-64
- 10 O'Sullivan GJ, Semba CP, Bittner CA. , et al. Endovascular management of iliac vein compression (May-Thurner) syndrome. J Vasc Interv Radiol 2000; 11 (07) 823-836
- 11 Patel NH, Stookey KR, Ketcham DB, Cragg AH. Endovascular management of acute extensive iliofemoral deep venous thrombosis caused by May-Thurner syndrome. J Vasc Interv Radiol 2000; 11 (10) 1297-1302
- 12 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
- 13 Raffini L, Raybagkar D, Cahill AM, Kaye R, Blumenstein M, Manno C. May-Thurner syndrome (iliac vein compression) and thrombosis in adolescents. Pediatr Blood Cancer 2006; 47 (06) 834-838
- 14 Goldman RE, Arendt VA, Kothary N. , et al. Endovascular management of May-Thurner syndrome in adolescents: a single-center experience. J Vasc Interv Radiol 2017; 28 (01) 71-77
- 15 Mitchell LG, Goldenberg NA, Male C, Kenet G, Monagle P, Nowak-Göttl U. ; Perinatal and Paediatric Haemostasis Subcommittee of the SSC of the ISTH. Definition of clinical efficacy and safety outcomes for clinical trials in deep venous thrombosis and pulmonary embolism in children. J Thromb Haemost 2011; 9 (09) 1856-1858
- 16 Kumar R, Rodriguez V, Matsumoto JM. , et al. Development and initial validation of a questionnaire to diagnose the presence and severity of post-thrombotic syndrome in children. Pediatr Blood Cancer 2012; 58 (04) 643-644
- 17 Kumar R, Rodriguez V, Matsumoto JM. , et al. Health-related quality of life in children and young adults with post-thrombotic syndrome: results from a cross-sectional study. Pediatr Blood Cancer 2014; 61 (03) 546-551
- 18 Steinberg JB, Jacocks MA. May-Thurner syndrome: a previously unreported variant. Ann Vasc Surg 1993; 7 (06) 577-581
- 19 Oguzkurt L, Tercan F, Ozkan U, Gulcan O. Iliac vein compression syndrome: outcome of endovascular treatment with long-term follow-up. Eur J Radiol 2008; 68 (03) 487-492
- 20 Hassell DR, Reifsteck JE, Harshfield DL, Ferris EJ. Unilateral left leg edema: a variation of the May-Thurner syndrome. Cardiovasc Intervent Radiol 1987; 10 (02) 89-91
- 21 Abboud G, Midulla M, Lions C. , et al. “Right-sided” May-Thurner syndrome. Cardiovasc Intervent Radiol 2010; 33 (05) 1056-1059
- 22 Ahmed HK, Hagspiel KD. Intravascular ultrasonographic findings in May-Thurner syndrome (iliac vein compression syndrome). J Ultrasound Med 2001; 20 (03) 251-256
- 23 Chung JW, Yoon CJ, Jung SI. , et al. Acute iliofemoral deep vein thrombosis: evaluation of underlying anatomic abnormalities by spiral CT venography. J Vasc Interv Radiol 2004; 15 (03) 249-256
- 24 Oguzkurt L, Tercan F, Pourbagher MA, Kizilkilic O, Turkoz R, Boyvat F. Computed tomography findings in 10 cases of iliac vein compression (May-Thurner) syndrome. Eur J Radiol 2005; 55 (03) 421-425
- 25 Gurel K, Gurel S, Karavas E, Buharalıoglu Y, Daglar B. Direct contrast-enhanced MR venography in the diagnosis of May-Thurner syndrome. Eur J Radiol 2011; 80 (02) 533-536
- 26 Jost CJ, Gloviczki P, Cherry Jr KJ. , et al. Surgical reconstruction of iliofemoral veins and the inferior vena cava for nonmalignant occlusive disease. J Vasc Surg 2001; 33 (02) 320-327 , discussion 327–328
- 27 Plate G, Einarsson E, Ohlin P, Jensen R, Qvarfordt P, Eklöf B. Thrombectomy with temporary arteriovenous fistula: the treatment of choice in acute iliofemoral venous thrombosis. J Vasc Surg 1984; 1 (06) 867-876
- 28 Garg N, Gloviczki P, Karimi KM. , et al. Factors affecting outcome of open and hybrid reconstructions for nonmalignant obstruction of iliofemoral veins and inferior vena cava. J Vasc Surg 2011; 53 (02) 383-393
- 29 Heijmen RH, Bollen TL, Duyndam DAC, Overtoom TT, Van Den Berg JC, Moll FL. Endovascular venous stenting in May-Thurner syndrome. J Cardiovasc Surg (Torino) 2001; 42 (01) 83-87
- 30 Bjarnason H, Kruse JR, Asinger DA. , et al. Iliofemoral deep venous thrombosis: safety and efficacy outcome during 5 years of catheter-directed thrombolytic therapy. J Vasc Interv Radiol 1997; 8 (03) 405-418
- 31 Verhaeghe R, Stockx L, Lacroix H, Vermylen J, Baert AL. Catheter-directed lysis of iliofemoral vein thrombosis with use of rt-PA. Eur Radiol 1997; 7 (07) 996-1001
- 32 Hurst DR, Forauer AR, Bloom JR, Greenfield LJ, Wakefield TW, Williams DM. Diagnosis and endovascular treatment of iliocaval compression syndrome. J Vasc Surg 2001; 34 (01) 106-113
- 33 Murphy EH, Davis CM, Journeycake JM, DeMuth RP, Arko FR. Symptomatic ileofemoral DVT after onset of oral contraceptive use in women with previously undiagnosed May-Thurner Syndrome. J Vasc Surg 2009; 49 (03) 697-703
- 34 Vedantham S, Grassi CJ, Ferral H. , et al; Technology Assessment Committee of the Society of Interventional Radiology. Reporting standards for endovascular treatment of lower extremity deep vein thrombosis. J Vasc Interv Radiol 2009; 20 (07) S391-S408
- 35 Neglén P, Hollis KC, Olivier J, Raju S. Stenting of the venous outflow in chronic venous disease: long-term stent-related outcome, clinical, and hemodynamic result. J Vasc Surg 2007; 46 (05) 979-990
- 36 Manco-Johnson MJ. Postthrombotic syndrome in children. Acta Haematol 2006; 115 (3-4): 207-213
- 37 Plate G, Eklöf B, Norgren L, Ohlin P, Dahlström JA. Venous thrombectomy for iliofemoral vein thrombosis--10-year results of a prospective randomised study. Eur J Vasc Endovasc Surg 1997; 14 (05) 367-374