Retrospective Study
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 21, 2017; 23(3): 486-495
Published online Jan 21, 2017. doi: 10.3748/wjg.v23.i3.486
Macro- and microcirculation patterns of intrahepatic blood flow changes in patients with hereditary hemorrhagic telangiectasia
Roland C Schelker, Ana P Barreiros, Christina Hart, Wolfgang Herr, Ernst-Michael Jung
Roland C Schelker, Christina Hart, Wolfgang Herr, Department of Internal Medicine III, Hematology and Oncology, University Hospital of Regensburg, 93051 Regensburg, Germany
Ana P Barreiros, Ernst-Michael Jung, Department of Radiology, University Hospital of Regensburg, 93051 Regensburg, Germany
Author contributions: Schelker RC conception and design, collection and/or assembly of data, analysis and interpretation, manuscript writing and revisions; Barreiros AP collection and/or assembly of data; Hart C manuscript writing and revisions; Herr W manuscript writing and revisions; Jung EM conception and design, analysis and interpretation, manuscript writing, final approval of manuscript.
Institutional review board statement: The study was reviewed and approved by the ethical committee of the University Hospital of Regensburg Institutional Review Board.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors indicate no potential conflicts of interest.
Data sharing statement: Technical appendix, statistical code, and dataset available from the corresponding author at Participants did not give informed consent for data sharing but the presented data are anonymized and risk of identification is very low.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Roland C Schelker, Department of Internal Medicine III, Hematology and Oncology, University Hospital of Regensburg, Franz-Josef-Strauss-Allee 11, 93051 Regensburg, Germany.
Telephone: +49-941-9445501 Fax: +49-941-9445514
Received: August 26, 2016
Peer-review started: August 29, 2016
First decision: September 28, 2016
Revised: October 7, 2016
Accepted: October 30, 2016
Article in press: October 31, 2016
Published online: January 21, 2017

To evaluated vascular dynamic processes in the liver of hereditary hemorrhagic telangiectasia (HHT) patients by ultrasound (US) considering quantitative analytic methods.


The imaging features on US and contrast-enhanced ultrasound (CEUS) in 18 patients diagnosed with HHT were retrospectively analyzed. Regarding CEUS, real-time contrast harmonic imaging and sulfur hexafluoride-filled microbubbles were used.


HVaMs were identified in all 18 patients. By US, the two major Caselitz criteria could be detected in 55.6% patients. "Color spots" were detected in 72.2% of the cases. Respecting sonographic grading criteria by Buscarini, grade 3 could be demonstrated most frequent (40%). By CEUS, all the patients showed quick and early hyperenhancement during the arterial phase. Significant lowest time to peak (TTP) and highest area under the curve (AUC) values were identified in the hepatic artery (TTP: 69.8%; AUC: 100%) and highest TTP and lowest AUC in the hepatic parenchyma and the portal vein.


For the first time we analyzed CEUS findings of a group of HHT patients regarding macro- and microcirculation. Our data demonstrate significant differences in TTP and AUC values in the four selected regions: hepatic artery, shunt region, portal vein and hepatic parenchyma.

Keywords: Hereditary hemorrhagic telangiectasia, Intrahepatic shunts, Contrast-enhanced ultrasound, Time-intensity-curve

Core tip: For the first time we analyzed contrast-enhanced ultrasound (CEUS) examination of a group of 18 hereditary hemorrhagic telangiectasia (HHT) patients regrading macro- and microcirculation. This new information could be used to sub-classify a high risk group of asymptomatic patients with therapeutic indication. With regard to the advent of new therapeutic agents, CEUS analysis can complete the required accurate cost-effective screening methods in HHT patients.