# High-Resolution Contrast-Enhanced Ultrasound with SRCEUS for Assessing the Intrahepatic Microvasculature and Shunts in Patients with Hereditary Haemorrhagic Teleangiectasia (Osler’s Disease)

## TL;DR

This study explores how high-resolution contrast-enhanced ultrasound can detect liver microvasculature changes and shunts in patients with Osler’s disease.

## Contribution

The study introduces SRCEUS as a novel diagnostic tool for detecting capillary-level changes and micro-shunts in hereditary haemorrhagic telangiectasia.

## Key findings

- SRCEUS detected echogenic foci in 5/10 cases of angiomas without late wash-out.
- Osler’s disease showed increased dilated capillaries and micro-shunts in both peripheral and central regions.
- Multiparametric ultrasound revealed elevated fibrosis, fat, and viscosity in a significant proportion of cases.

## Abstract

The aim of this retrospective clinical pilot study is to evaluate multiparametric ultrasound liver parenchyma assessments in the diagnosis of Osler’s disease, and to detect micro-shunts using SRCEUS with quantifications at the capillary level. Material/Method: All examinations were performed by an experienced examiner with a multi-frequency probe on a high-resolution matrix ultrasound device (SC 7-1U), convex probe (Mindray A 20), and were stored digitally in the PACS system. Vascular ultrasound was performed using colour-coded Doppler ultrasound (CCDS) and ultrasound microangiography (UMA). The recent M-Ref tool was utilised for the purpose of liver tissue characterisation, encompassing the domains of shear wave elastography, fat evaluation, and viscosity. Dynamic CEUS, HiFR CEUS, and SR CEUS were performed after the intravenous bolus injection of 1–2.4 mL of ultrasound contrast agent (SonoVue®). Measurements of SR CEUS capillary changes were performed independently by PACS-stored digital cine loops up to 5 s. Results: In the context of angiomas or haemangiomas, the initial contrast enhancement of echogenic or almost echogenic foci within 25 s without late wash-out was observed in 5/10 cases. In the evaluation of microvasculature, the presence of capsule-proximal shunts in Osler’s disease was observed, resulting in the identification of increased numbers of dilated capillaries within both peripheral and central shunts. In the control group, general liver tissue changes (20 cases) were observed in instances of inflammation (3/20 cases), peripherally in 4/20 cases with micro-shunts in altered parenchyma. In the context of multiparametric ultrasound, 16 out of 30 cases exhibited elevated fibrosis values, with a maximum recorded as high as 1.7 m/s, and in 13 out of 30 cases, there was an increase in fat values up to 0.65 dB/cm/MHz, indicative of moderate steatosis. Additionally, in seven cases, there was an increase in viscosity values up to 2.7 Pa·s, suggesting reactive changes. Conclusions: Recent advancements in medical imaging technology, specifically SR CEUS contrast ultrasound imaging, have led to the development of novel diagnostic tools that facilitate the evaluation of tissue and haemodynamic changes, in addition to capillary alterations, associated with Osler’s disease.