Current diagnostic and therapeutic standard of iliac vein compression syndrome (May-Thurner syndrome) in children, adolescents and young adults. A survey among national thrombosis experts

Introduction Iliac vein compression syndrome (May-Thurner Syndrome) is an anatomic variant with chronic pulsatile compression of the left common iliac vein by the overlying right common iliac artery against lumbar vertebrae. Reduced venous backflow and endothelial damage result in a predisposition for left-sided deep vein thromboses. The prevalence of iliac vein compression syndrome may be underestimated as the sensitivity of colour doppler sonography to screen for iliac compression is low. Therapeutic options comprise anticoagulation, thrombectomy and intravascular stent. The aim of this survey was to assess the current practice in Germany to develop a standardized diagnostic and therapeutic approach.

Method Our interdisciplinary team (pediatric hemostaseology, radiology, vascular surgery) designed an online survey consisting of 11 questions which we distributed via the mailing list to the members of the German Society of Thrombosis and Hemostasis.

Fig. 1  Centre characterization; Characterization of centres participating in the survey in %; most centres are hospitals (pediatrics/ pediatric hemostaseology, internal medicine/ angiology, vascular surgery) and outpatient practices (general practice, pediatric practice including hemostaseology/ pediatric oncology and hematology, general practice including hemostaseology) along with a few others (not shown: pediatric cardiology, transfusion medicine/ hemostaseology, internal medicine/ rheumatology).

Results Between July and October 2022, 33 questionnaires were returned. Most participating centres are hospitals ([Fig. 1]). 52% treat patients <18 years, 21% adults and 27% all age groups. Numbers of annually treated thromboses in patients <25 years range from 1-5 (26%) to >30 (13%). Centres treating adult patients reported higher patient numbers. Main diagnostic tools used for deep vein thrombosis (DVT) are clinical/ laboratory workup and doppler sonography, only 53% of centres report the use of MRI ([Fig. 2]). DVT is treated by therapeutic anticoagulation (84%), systemic fibrinolysis (13%), interventional thrombectomy (39%), catheter lysis/ ultrasound directed methods (32%) or surgical thrombectomy (10%). Screening for iliac vein compression syndrome is performed by 25% of centres (always 7%, in case of typical clinical signs 4%, in case of left-sided DVT 4%, in case of iliac vein compression 7%). Treatment for iliac vein compression syndrome are anticoagulation (65%), balloon angioplasty (13%) or stent/ AV fistula (32%). Choice of treatment is highly individualized and centre specific. Interventional treatment is administered depending on thrombus size (68%), age of thrombosis (65%), and thrombus localization (71%), for lack of contraindications (35%) or to avoid post-thrombotic syndrome (35%). Half of participating centres identified as specialized treatment centres, in 36% patients are referred to the centres [1] [2] [3].

Fig. 2  Diagnostic methods for diagnosis of a deep vein thrombosis (%); Diagnostic tools used for diagnosis of DVT in all centres (blue bars), centres treating adult and pediatric patients (grey) and pediatric centres (yellow), shown in %. Main diagnostic methods are clinical/ laboratory workup and doppler sonography. Only 53%-60% chose MRI and 65-68% MRI-angiogram. CT and CT-angiogram are done less frequently, and very rarely in pediatric centres. Conventional angiography and phlebography are used by 4-9%. None of the centres opt for intravascular sonography.

Conclusion The participating centres use various diagnostic and therapeutic options for young patients with iliac vein compression syndrome. As expected, approaches differ between centres reflecting the limited experience with this patient group. Only 25% of centres systematically screen for iliac vein compression. To optimize care it is crucial to compare different therapeutic options currently used and follow up on outcome. Next steps are to develop a standardized diagnostic approach and therapeutic algorithm within our interdisciplinary team and the working group Pediatrics of the German Society of Thrombosis and Hemostasis.

Publikationsverlauf

Artikel online veröffentlicht:
20. Februar 2023

• References

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