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.
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: firstname.lastname@example.org.
SUPPLEMENTARY MATERIAL accompanies this paper at http://links.lww.com/AJG/A81
Received August 05, 2018
Accepted January 28, 2019
Online date: March 13, 2019