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Safety of Endoscopy in Heritable Connective Tissue Disorders

Kilaru, Saikiran M. MD1; Mukamal, Kenneth J. MD, MPH2; Nee, Judy W. MD2; Oza, Sveta S. MD2; Lembo, Anthony J. MD2; Wolf, Jacqueline L. MD2

American Journal of Gastroenterology: August 2019 - Volume 114 - Issue 8 - p 1343–1345
doi: 10.14309/ajg.0000000000000189

Introduction: Little is known about the gastrointestinal manifestations or safety of endoscopy among patients with heritable connective tissue disorders such as Marfan syndrome or Ehlers-Danlos syndrome (EDS).

Methods: We conducted an electronic cross-sectional survey nested within preexisting registries of patients with heritable connective tissue disorders and examined self-reported rates of endoscopic complications.

Results: The rate of endoscopy-related perforation was 9.4% (95% confidence interval 2.0%–25.0%) among individuals with vascular EDS, <1% in classical and hypermobility-type EDS, and zero in Marfan syndrome (P < 0.001). Spontaneous intestinal perforation was also significantly higher in the vascular EDS group.

DISCUSSION: Clinicians should consider noninvasive screening methods for patients with vascular EDS.

1New York University School of Medicine, New York, New York, USA;

2Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.

Correspondence: Jacqueline L. Wolf, MD. E-mail:

SUPPLEMENTARY MATERIAL accompanies this paper at

Received August 05, 2018

Accepted January 28, 2019

Online date: March 13, 2019

© The American College of Gastroenterology 2019. All Rights Reserved.
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