Ehlers Danlos syndrome and gastrointestinal manifestations: a 20‐year experience at Mayo Clinic

Abstract

EDS HM and other subtypes should be considered in patients with chronic functional GI symptoms and abdominal vascular lesions.

Increased Need for Gastrointestinal Surgery and Increased Risk of Surgery-Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review

“…Two studies presented a wrong patient group, 5 reported outcomes related to vascular surgery, and 3 held vascular interventions, and were excluded. The remaining 11 studies met the study eligibility criteria, of which 2 studies held the same 100 patients as a continuum [21,22] , and altogether summed 1,567 patients [4,13,21,29] . In total, 1,312 patients had the diagnosis confirmed by Villefranche criteria, while the remaining patients were retrieved from national EDSs foundations and health clinics with no reported method of diagnosis.…”

“…Acute complications were most prevalent in the vascular subtype, both primary manifestations that needed surgical intervention and secondary post-surgical complications. Functional GI symptoms were more prevalent in the hypermobility type and to a lesser extent in the classic type [4] . Reduced life expectancy could not be proven as a general feature of EDSs.…”

“…Interestingly, when undergoing colonoscopy, a higher number of up to 10% were found with GI diverticulosis, indicating that the prevalence might be significantly higher if all patients with EDSs were examined radiologically or endoscopically [4] .…”

“…Within the different subtypes, the included studies reported the highest incidence in the hypermobility type. Notably, rectal prolapse was not shown to be specifically associated with vascular EDS, which holds the highest rate for ruptured GI structures [4] . No data was reported on mortality, preferred surgical technique for managing rectal prolapse, or complications secondary to surgical intervention.…”

“…Data showed that GI surgery had been performed in 10% of all patients with EDSs, but with a higher incidence of up to 33% of the vascular subtype [4] . For this group, the need for surgery other than GI surgery, both surgery for arterial dissection or rupture, spontaneous bowel perforation or organ rupture, altogether constituted as high a figure as 68% [13] .…”