Skip to main content
2025 ASCRS Annual Scientific Meeting Main banner
Final Program


Visit ASCRS U
Icon Legend
This session is not in your schedule.
This session is in your schedule. Click again to remove it.
Presentation Icons
CME
CME
Invitation/Ticketed Event
Invitation/Ticketed Event
Invited Lectureship
Invited Lectureship
Award Recipient
Award Recipient
Self-Assessment
Self-Assessment
Social
Social
Industry
Industry
Back

Session: ePoster: Benign Abdominal 11 (Sat315)

EP107 - Systematic Literature Review of Intestinal Perforations in Vascular Ehlers-Danlos Syndrome

Saturday, May 10, 2025
3:58 PM – 4:03 PM PT
Location: Monitor 1, Sails Pavilion, Upper Level

    ePoster Presenter(s)

  • Maura Walsh, PGY-4

    Surgical resident
    Oregon Health Sciences University
    Portland, OR, United States

    All of the relevant financial relationships listed below have been mitigated.
    This individual has no financial relationships with ineligible companies.

    • Author(s)

  • Cynthia A. Araradian, MD

    Resident Physician
    OHSU
    Portland, OR, United States

    All of the relevant financial relationships listed below have been mitigated.
    This individual has no financial relationships with ineligible companies.

  • MM

    Majdeddin MohammedAli, MD

    Resident, General Surgery
    The Brooklyn Hospital Center, Brooklyn, NY
    Brooklyn, New York, United States

    Disclosure information not submitted.

  • Sandy H. Fang, MD

    Associate Professor
    Oregon Health and Science University
    Portland, Oregon, United States

    All of the relevant financial relationships listed below have been mitigated.
    This individual has no financial relationships with ineligible companies.

    • Senior Author(s)

  • SS

    Sherene Shalhub, MD MPH

    Professor and Division Head
    OREGON HEALTH & SCIENCE UNIVERSITY
    PORTLAND, Oregon, United States

    All of the relevant financial relationships listed below have been mitigated.
    This individual has no financial relationships with ineligible companies.

All of the relevant financial relationships listed below have been mitigated.
Maura Walsh, PGY-4: This individual has no financial relationships with ineligible companies.
Cynthia A. Araradian, MD: This individual has no financial relationships with ineligible companies.
Sandy H. Fang, MD: This individual has no financial relationships with ineligible companies.
Sherene Shalhub, MD MPH: This individual has no financial relationships with ineligible companies.
Image or Table (Do Not add text here):

Purpose/Background: Vascular Ehlers-Danlos Syndrome (vEDS) is an extremely rare autosomal dominant genetic connective tissue disorder caused by the pathogenic variant COL3A1 gene. The phenotypic manifestation of vEDS is characterized by tissue fragility, which results in vascular dissection and rupture, as well as hollow viscus perforation. The most morbid gastrointestinal sequelae of vEDS is intestinal perforation. VEDS is challenging to study because it affects 2/100,000 individuals and accounts for 5% of EDS. The purpose of this meta-analysis is to statistically quantify the risk of vEDS-associated intestinal perforation and evaluate surgical outcomes to determine best practices in care.

Methods/Interventions: A systematic literature search was performed using Pubmed, Cinahl, Cochrane, and EMBASE in August 2023 identifying vEDS-related intestinal perforation publications since the 1980s. The initial search produced 615 articles with 530 articles screened after the removal of duplicates. Two independent reviewers and a third independent reviewer for conflict resolution performed data extraction, risk of bias assessments, and narrative synthesis. Certainty of evidence was assessed by the Grading Recommendations, Assessment, Development and Evaluations (GRADE) framework. All data were reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).

Results/Outcomes: Five non-randomized studies were selected for the meta-analysis and included a total of 854 patients with vEDS. The studies were published between 2000-2021 and included patients with genetically confirmed vEDS. Statistical analysis was performed with R to calculate the colonic perforation rate, intestinal perforation rate and reperforation rate for this patient population. The pooled colonic perforation rate was 18% (n=157), small intestinal perforation rate 3% (n=14) and reperforation rate 37% (n=43). The events of a colonic or small bowel perforation often preceded the diagnosis of vEDS which was noted in 3 of the papers and estimated to be near 75%.

Conclusion/Discussion: While patients with vEDS have high colonic perforation and subsequent reperforation risks, the majority of vEDS diagnoses are not made until after the inciting event of bowel perforation. No consensus guidelines exist for the surgical management of vEDS-related bowel perforations. Given the rarity of disease, data is limited on this study population and needs to be further studied and validated.