May-Thurner syndrome in patients with postural orthostatic tachycardia syndrome and Ehlers-Danlos syndrome: a case series

Abstract

Background Postural orthostatic tachycardia syndrome (POTS), Ehlers-Danlos syndrome (EDS), and May-Thurner syndrome (MTS) are three syndromes that are often misdiagnosed or underdiagnosed. The true prevalence of these syndromes may be higher than currently reported. The following case series is the first to report a three-way association between POTS, EDS, and MTS. Case Summary We describe three patients with concomitant POTS, EDS, and MTS. Although abdominopelvic vasculature evaluation can be difficult via conventional imaging techniques, we present the use of novel dynamic contrast-enhanced magnetic resonance angiography with Differential Subsampling with Cartesian Ordering (DISCO) and Four-Dimensional Flow magnetic resonance imaging to aid vasculature evaluation and the diagnosis of MTS. Two patients underwent left common iliac vein (LCIV) stenting to treat MTS, experiencing significant improvement in their POTS symptoms and quality of life. Discussion EDS, POTS, and MTS may interact synergistically to exacerbate symptoms. Patients with EDS should be evaluated for possible POTS and pelvic venous complications. LCIV stenting for MTS can mitigate POTS symptoms by decreasing lower extremity venous pooling and should be considered in this patient population. Further research is needed to understand the exact mechanism and intricacies of this syndrome triad.

Improvement in chronic pelvic pain, orthostatic intolerance and interstitial cystitis symptoms after treatment of pelvic vein insufficiency

“…Orthostatic intolerance/dysautonomia is also well known to be related to EDS, 14,48,49 findings supported here. 12,40 CPP and vulvodynia have also been associated with EDS and DA by some authors. 36,37,39,50 Of all present findings, the treatment effect after pelvic venous interventions on orthostatic intolerance/dysautonomia is the most remarkable.…”

“…The comorbidities of VO-CPP analyzed here have been occasionally linked to CPP, [35][36][37][38][39] but rarely to VO-CPP. 12,40,41 The omnibus questionnaire combining the IPPS, the validated surveys PUF and OHQ, and a modified Rome III, found a high prevalence of symptoms of IC, DA, and IBS, where roughly half the group scored in the severe range (64, 49, 40%, respectively). These particular values might reflect referral bias, although they are very similar to the recent survey findings from an Internet support group for PCS.…”

“…Previously, a three-patient case report suggested pelvic veins, EDS, and POTS were related. 51 POTS and DA are thought of as being of unknown cause, and treatment has been symptomatic. 46 It has been known that DA is linked to blood volume and pressure sequestered below the waist; EDS causing increased capacitance also has been implicated, along with autonomic system dysfunction and compensatory sympathetic overdrive.…”

“…The fact that "dysautonomia" is present commonly is worrisome since it may be having a severe disabling effect on some VO-CPP patients. 51 The PUF dysuria scale characterizes pelvic pain from a urologist's point of view and the IPPS from that of a gynecologist. This situation reflects the various specialtyspecific definitions of pelvic pain syndromes.…”

“…The fact that “dysautonomia” is present commonly is worrisome since it may be having a severe disabling effect on some VO-CPP patients. 51…”