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Hereditary hemorrhagic telangiectasia: how to efficiently detect hepatic abnormalities using ultrasonography

  • Review Article–Gastroenterology
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Abstract

Introduction

Hereditary hemorrhagic telangiectasia (HHT) is a multiorgan genetic angiodysplastic affection characterized by visceral vascular malformations. It affects mainly the brains, lungs, gastrointestinal tract, and nasal mucosa. Unlike those organs, hepatic involvement, although very frequently occurring, is insufficiently recognized, mainly because of the complex vascular structure of this organ. Thus, treating HHT patients requires a solid understanding of these hepatic anomalies. It is especially important for any general clinicians to be able to recognize clinical findings in HHT, which leads to a high suspicion of HHT and have an index of suspicion for liver abnormalities of HHT. For this purpose, keen awareness of clinical as well as hepatic sonographic (US) findings is paramount.

Aim

The aim of this review is to summarize previously reported findings on the hepatic US through a thorough analysis of related articles, and to (a) determine the role of US in the diagnosis of hepatic involvement in HHT patients and (b) propose the most simple and easy way to detect HHT-related abnormalities during routine US examinations.

Conclusion

Hepatic US serves to diagnose the detailed complex hepatic changes typical of HHT, and contributes to increased diagnostic confidence of hepatic changes in HHT patients, with the most simple way not to overlook HHT-related abnormalities being to find hepatic artery dilatation.

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Abbreviations

US:

Ultrasonography

HHT:

Hereditary hemorrhagic telangiectasia

AV shunt:

Arteriovenous shunt

AP shunt:

Arterioportal shunt

PV shunt:

Portovenous shunt

VVshunt:

Venovenous shunt

AVM:

Arteriovenous malformation

SMI:

Superb microvascular imaging

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Authors and Affiliations

  1. Department of Gastroenterology, Yokote Municipal Hospital, 5-31 Negishi-cho, Yokote, Akita, 013-8602, Japan

    Hiroko Naganuma

  2. Center of Diagnostic Ultrasound, Akita Red Cross Hospital, Akita, Japan

    Hideaki Ishida

  3. Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Iwate, Japan

    Hidekatsu Kuroda

  4. Department of Gastroenterology, Nayoro City General Hospital, Hokkaido, Japan

    Yasuaki Suzuki

  5. Department of Gastroenterology and Hepatology, Nihon University Hospital, Tokyo, Japan

    Masahiro Ogawa

Authors
  1. Hiroko Naganuma
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  2. Hideaki Ishida
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  3. Hidekatsu Kuroda
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  4. Yasuaki Suzuki
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  5. Masahiro Ogawa
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Corresponding author

Correspondence to Hiroko Naganuma.

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We have no potential conflicts of interest.

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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008 (5).

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Informed consent was obtained from all patients for being included in the study.

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Naganuma, H., Ishida, H., Kuroda, H. et al. Hereditary hemorrhagic telangiectasia: how to efficiently detect hepatic abnormalities using ultrasonography. J Med Ultrasonics 47, 421–433 (2020). https://doi.org/10.1007/s10396-020-01022-w

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