Restricted accessResearch articleFirst published online 2025
Comparative assessment of left common iliac vein compression in patients with hypermobile Ehlers-Danlos syndrome,hypermobility spectrum disorder and healthy controls – A retrospective single-centre study
Heritable connective tissue disorders (HCTDs), including hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobile spectrum disorder (HSD), have complex clinical manifestations, but the prevalence of left common iliac vein (LCIV) compression in these groups is not well known. This study aimed to compare the prevalence of significant LCIV compression in patients with hEDS, HSD, and healthy controls.
Methods
This retrospective, IRB-approved, single-center study analyzed 109 patients (25 hEDS, 64 HSD, 20 controls) who underwent contrast-enhanced abdominal and pelvic CT imaging from 2020 to 2023. LCIV compression was measured on axial CT, with >50% luminal narrowing defined as significant. Statistical analyses included chi-square tests, odds ratios, and t-tests. Interrater reliability was evaluated using intraclass correlation coefficients.
Results
Significant LCIV compression occurred in 68.0% of hEDS, 65.6% of HSD, and 35.0% of controls (p = 0.034). Both hEDS (p = 0.027) and HSD (p = 0.015) groups had significantly higher compression rates than controls, with no statistically significant difference between the two clinical groups (p = 0.831). Odds of compression were nearly fourfold greater in hEDS (OR 3.95) and HSD (OR 3.54) compared to controls.
Conclusion
LCIV compression is significantly more prevalent in patients with hEDS and HSD than in healthy controls, warranting further investigation of its clinical significance.
BascomRSchubartJRMillsS, et al.Heritable disorders of connective tissue: description of a data repository and initial cohort characterization. Am J Med Genet2019; 179(4): 552–560.
CastoriMCamerotaFCellettiC, et al.Ehlers-Danlos syndrome hypermobility type and the excess of affected females: possible mechanisms and perspectives. Am J Med Genet2010; 152A(9): 2406–2408.
7.
DarakjianAABhutaniMFairweatherD, et al.Similarities and differences in self-reported symptoms and comorbidities between hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders. Rheumatol Adv Pract2024; 8(4): rkae134.
8.
HertelABlackWRMalloy WaltonL, et al.Cardiovascular symptoms, dysautonomia, and quality of life in adult and pediatric patients with hypermobile Ehlers-Danlos syndrome: a brief review. Curr Cardiol Rev2024; 20: E240124226070.
9.
OrmistonCKPadillaEVanDT, et al.May-thurner syndrome in patients with postural orthostatic tachycardia syndrome and Ehlers-Danlos syndrome: a case series. Eur Heart J Case Rep2022; 6(4): ytac161.
10.
ClarkNLJohnsonMRanganA, et al.The biopsychosocial impact of hypermobility spectrum disorders in adults: a scoping review. Rheumatol Int2023; 43(6): 985–1014.
11.
SantoshiRKNLakhanpalSSatwahV, et al.Iliac vein stenosis is an underdiagnosed cause of pelvic venous insufficiency. J Vasc Surg Venous Lymphat Disord2018; 6(2): 202–211.
12.
KnuttinenMGZurcherKSKhuranaN, et al.Imaging findings of pelvic venous insufficiency in patients with postural orthostatic tachycardia syndrome. Phlebology2021; 36(1): 32–37.
13.
OguzkurtLOzkanUUlusanS, et al.Compression of the left common iliac vein in asymptomatic subjects and patients with left iliofemoral deep vein thrombosis. J Vasc Intervent Radiol2008; 19(3): 366–371.
14.
ZhuQYangLZhuH, et al.Prevalence of left iliac vein compression in an asymptomatic population and patients with left iliofemoral deep vein thrombosis: a multicenter cross-sectional study in southern China. Phlebology2022; 37(8): 602–609.
15.
GongMJiangRHeX, et al.Relationship between left moderate or severe common iliac vein compression and occurrence of involved iliac vein thrombosis in patients with infrainguinal deep vein thrombosis. Cardiovasc Interv Radiol2025; 48(3): 304–313.
16.
LiuJLiuPXiaK, et al.Iliac vein compression syndrome (IVCS): an under-recognized risk factor for left-sided deep venous thrombosis (DVT) in old hip fracture patients. Med Sci Monit2017; 23: 2078–2082.
17.
WuMKLuoXYZhangFX. Incidence and risk factors of deep venous thrombosis in asymptomatic iliac vein compression: a prospective cohort study. Chin Med J2016; 129(18): 2149–2152.
18.
BergenMAWallKCKimCY, et al.Occult left common iliac vein compression increases postoperative venous thromboembolism risk following total hip arthroplasty. J Arthroplast2019; 34(2): 375–378.
19.
KibbeMRUjikiMGoodwinAL, et al.Iliac vein compression in an asymptomatic patient population. J Vasc Surg2004; 39(5): 937–943.
20.
WangKSiegelLBetancourtA, et al.Frequency of left common iliac vein compression in asymptomatic adolescents and young adults. J Vasc Surg Venous Lymphat Disord2025; 13: 102282.
21.
MalfaitFFrancomanoCByersP, et al.The 2017 international classification of the ehlers-danlos syndromes. Am J Med Genet C Semin Med Genet2017; 175(1): 8–26.
22.
FairweatherDBrunoKADarakjianAA, et al.High overlap in patients diagnosed with hypermobile Ehlers-Danlos syndrome or hypermobile spectrum disorders with fibromyalgia and 40 self-reported symptoms and comorbidities. Front Med2023; 10: 1096180.
23.
GagnePJTaharaRWFastabendCP, et al.Venography versus intravascular ultrasound for diagnosing and treating iliofemoral vein obstruction. J Vasc Surg Venous Lymphat Disord2017; 5: 678–687.
24.
GagnePJGasparisABlackS, et al.Analysis of threshold stenosis by multiplanar venogram and intravascular ultrasound examination for predicting clinical improvement after iliofemoral vein stenting in the VIDIO trial. J Vasc Surg Venous Lymphat Disord2018; 6: 48–56.e1.
25.
ShammasNWShammasGAJones-MillerS, et al.Predicting iliac vein compression with computed tomography angiography and venography: correlation with intravascular ultrasound. J Invasive Cardiol2018; 30: 452–455.
26.
RossiFHKambaraAMRodriguesTO, et al.Comparison of computed tomography venography and intravascular ultrasound in screening and classification of iliac vein obstruction in patients with chronic venous disease. J Vasc Surg Venous Lymphat Disord2020; 8(3): 413–422.
27.
PoyyamoliSMehtaPCherianM, et al.May-thurner syndrome. Cardiovasc Diagn Ther2021; 11(5): 1104–1111.
28.
SaleemTRajuS. Comparison of intravascular ultrasound and multidimensional contrast imaging modalities for characterization of chronic occlusive iliofemoral venous disease: a systematic review. J Vasc Surg Venous Lymphat Disord2021; 9(6): 1545–1556.e2.
29.
DwivediASinghSNSharmaA, et al.A systematic review of radiological diagnosis and management of may-thurner syndrome. J Pharm BioAllied Sci2024; 16(Suppl 2): S1012–S1016.
30.
CastoriMTinkleBLevyH, et al.A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet2017; 175(1): 148–157.
31.
FarinaRGozzoCFotiPV, et al.A man with the rare simultaneous combination of three abdominal vascular compression syndromes: median arcuate ligament syndrome, superior mesenteric artery syndrome, and nutcracker syndrome. Radiol Case Rep2021; 16(6): 1264–1270.
32.
NunnRHenryJSlesserAA, et al.A model example: coexisting superior mesenteric artery syndrome and the nutcracker phenomenon. Case Rep Surg2015; 2015: 649469.
33.
TiralongoFGaliotoFDistefanoG, et al.Anterior and posterior nutcracker syndrome combined with may-thurner syndrome: first report of this unique case. Diagnostics2023; 13(8): 1433.
34.
MohammedAZinabuSWMichaelMB. Navigating complexities: a case of multiple abdominopelvic vascular compression syndromes in Ehlers-Danlos syndrome. Cureus2024; 16(7): e63848.
35.
JayarajABuckWKnightA, et al.Impact of degree of stenosis in may-thurner syndrome on iliac vein stenting. J Vasc Surg Venous Lymphat Disord2019; 7(2): 195–202.
36.
MetzgerPBRossiFHKambaraAM, et al.Criteria for detecting significant chronic iliac venous obstructions with duplex ultrasound. J Vasc Surg Venous Lymphat Disord2016; 4(1): 18–27.
37.
OtaHTakaseKRikimaruH, et al.Quantitative vascular measurements in arterial occlusive disease. Radiographics2005; 25(5): 1141–1158.
