This article is an extension of either a plenary guest speaker lecture or an abstract presented at the EDS ECHO Conference on the Ehlers–Danlos syndromes held on October 2–3, 2020. The Editors-in-Chief of this journal affirm that this article was evaluated editorially and rigorously edited by the expert Guest Editors for this issue, Dr. Hakim, Dr. Francomano, and Dr. Tinkle and was not anonymously peer reviewed.
Case reports and systematic studies of the most common hypermobility-related disorders, hypermobile Ehlers–Danlos syndrome (hEDS), and hypermobility spectrum disorder (HSD) typically describe gastroenterological symptoms and complaints attributed to structural malfunction, autonomic dysfunction, or inflammation of the gastrointestinal tract. However, abdominal compression syndromes (CS) may also contribute to pain and dysfunction in these individuals and be the leading pathology given symptoms significantly reduce or cease after decompressive surgery. Arising not only in the abdomen and causing pain (median arcuate ligament syndrome [MALS] and superior mesenteric artery syndrome [SMAS]), CS also occur in the retroperitoneum and the pelvis (nutcracker syndrome and May–Thurner syndrome), these latter conditions causing chronic pelvic congestion syndrome (PCS). Here, we report primarily on our experience of the assessment and management of MALS and SMAS in a cohort of cases with a surprising prevalence of HSD and hEDS. To our knowledge, this is the first cohort report of its kind in hEDS, HSD, and CS. We recommend that CS are considered in hEDS and HSD individuals with gastrointestinal and other painful complaints within the “belt” area. These CS can be identified using functional ultrasound duplex examination in experienced hands, and in appropriate cases stabilizing surgery can substantially improve quality of life.
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