Safety of Endoscopy in Heritable Connective Tissue Disorders.

Kilaru, Saikiran M; Mukamal, Kenneth J; Nee, Judy W; Oza, Sveta S; Lembo, Anthony J; Wolf, Jacqueline L · Am J Gastroenterol · 2019

cross_sectional · Level IV

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Abstract

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

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