The Investigation and Management of the Abdominopelvic Vascular Compression Syndromes in Patients with Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder

Abdominopelvic Vascular Compression Syndrome(s) (VCS) are rare disorders with diverse symptoms that appear to occur more frequently in patients with Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD). The reported associations between EDS/HSD, Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS) further complicate the diagnosis and management of the VCS in this specific patient population. This review summarises the established literature on this complex topic, highlighting these relationships, with the aim to propose a framework for recognising and managing VCS among patients with EDS/HSD. Given the limited body of literature on this topic, we also aim to underscore the need for further research within this specific patient population. A PRISMA-guided systematic review was conducted using PubMed and Ovid/Medline databases. VCS included Median Arcuate Ligament Syndrome (MALS), Superior Mesenteric Artery Syndrome (SMAS), Nutcracker Syndrome (NCS), and May-Thurner Syndrome (MTS). Given the limited number of studies, small cohort studies and case reports/series were also reviewed. Of 183 screened studies, 62 met the inclusion criteria. Only six studies directly addressed the VCS in EDS/HSD. Five discussed an EDS-POTS association, two described links between MCAS, POTS, and EDS, and five associated POTS with VCS. Only one study explored all four conditions. Evidence suggests an association between EDS/HSD, VCS, POTS, and MCAS but remains limited. Underdiagnosis and delayed treatment are common and underscore the need for multi-disciplinary care. Invasive imaging and interventions appear generally safe in EDS/HSD, excluding vascular EDS, yet robust safety and outcome data and tailored diagnostic or treatment algorithms are lacking and require further investigation.