BACKGROUND/AIMS: Ehlers-Danlos syndromes (EDSs) constitute a rare group of 
inherited connective tissue diseases, characterized by multisystemic 
manifestations and general tissue fragility. Most severe complications include 
vascular and gastrointestinal (GI) emergencies requiring acute surgery. The 
purpose of this systematic review was to assess the causes of GI-related surgery 
and related mortality and morbidity in patients with EDSs.
METHODS: A systematic search was conducted in PubMed, Embase, and Scopus to 
identify relevant studies. Preferred Reporting Items for Systematic Reviews and 
Meta-Analysis guidelines for systematic reviews were followed. According to 
eligibility criteria, data were extracted and systematically screened by 2 
authors.
RESULTS: Screening process identified 11 studies with a total of 1,567 patients. 
Findings indicated that patients with EDSs had a higher occurrence of surgery 
demanding GI manifestations, including perforation, hemorrhage, rupture of 
intra-abdominal organs, and rectal prolapse. Most affected was the vascular 
subtype, of which up to 33% underwent GI surgery and suffered from a lowered 
average life expectancy of 48 years (range 6-78). Secondary complications of 
surgery were common in all patients with EDSs.
CONCLUSION: Studies suggested that patients with EDSs present an increased need 
for GI surgery, but also an increased risk of surgery-related complications, 
most predominantly seen in the vascular subtype.