Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia: Long-term results of endoscopic treatment

Guido Manfredi; Stefano Francesco Crinò; Saverio Alicante; Samanta Romeo; Roberto Berté; Silvia Gandolfi; Angelo Spinazzola; Michela Fiini; Pierangelo Forner; Elisabetta Buscarini

doi:10.1055/a-2190-9303

Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia: Long-term results of endoscopic treatment

Endosc Int Open. 2023 Dec 15;11(12):E1145-E1152. doi: 10.1055/a-2190-9303. eCollection 2023 Dec.

Affiliations

¹ Gastroenterology and Endoscopy Department, Center of reference VASCERN HHT, Maggiore Hospital Crema, Crema, Italy.
² Digestive Endoscopy Unit, University of Verona, Italy, Verona, Italy.
³ Radiology Department, Maggiore Hospital Crema, Crema, Italy.
⁴ Cardiology Department, Maggiore Hospital Crema, Crema, Italy.
⁵ ENT Department, Maggiore Hospital Crema, Crema, Italy.

Abstract

Background and study aims This longitudinal prospective study evaluated the long-term outcome of endoscopic treatment of gastrointestinal bleeding in hereditary hemorrhagic telangiectases (HHT), its safety and outcome predictors. Patients and methods Consecutive patients with HHT and either anemia disproportionate to epistaxis or overt gastrointestinal bleeding received endoscopic treatment of gastrointestinal telangiectases with argon plasma coagulation (APC). Hemoglobin levels and transfusion requirements were evaluated before and after treatment. Treatment effectiveness was classified as: 1) complete: hemoglobin level during the follow-up ≥9 g/dL; 2) complete with recurrence: hemoglobin ≥9 g/dL for at least 12 months with subsequent drop to <9 g/dL; or 3) absent: no improvement of hemoglobin level. Adverse events (AEs) were classified as mild, moderate, severe or fatal. Correlations were searched between treatment outcome and demographic/genetic characteristics, number, size and site of telangiectases, and hepatic arterio-venous malformations grade. Results Forty-seven patients with HHT were enrolled. At median follow-up of 134 months (range 20-243 months), 41 of 47 patients showed treatment response (complete or with recurrence) after one (14/47) or more (27/47) endoscopic treatments. Median hemoglobin levels were 7.0 g/dL and 11.9 g/dL at baseline and at the end of follow-up, respectively. Transfusion requirement decreased from 22.8 to 7.3 red cell unit/year. A higher baseline number of telangiectases was associated with a lower chance of response ( P =0.008). Only one severe AE (0.4%, jejunal perforation) was recorded. Conclusions Endoscopic treatment of gastrointestinal teleangiectases for gastrointestinal bleeding in patients with HHT is effective in the long term and safe.

Keywords: RFA and ablative methods; Small bowel endoscopy; Small intestinal bleeding.

The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Grants and funding

This study has been funded with an unrestricted grant by Italian Association for Hereditary Hemorrhagic Telangiectasia "Onilde Carini". The funding body had no role in the design of the study and collection, analysis, and interpretation of data nor in writing the manuscript.