Summary

Vascular diseases of the liver include portal vein thrombosis (with or without cirrhosis), portosinusoidal vascular disorder, Budd-Chiari syndrome, sinusoidal obstruction syndrome, non-obstructive sinusoidal dilatation and peliosis, splanchnic artery aneurysms, and hepatic arteriovenous fistulas. Except for portal vein thrombosis in cirrhosis, these are all rare conditions. Since the last Clinical Practice Guidelines were issued by the European Association for the Study of the Liver in 2016, much data has been published on the diagnosis and management – medical and interventional – of patients with vascular liver diseases. Based on a thorough review of the relevant literature, recommendations are provided to address key clinical dilemmas. The document emphasises personalised care, considering individual risk factors and clinical presentation. Multidisciplinary management involving hepatologists, haematologists, pathologists, interventional radiologists and surgeons is essential in this area. Our aim is to provide guidance on the management of patients with vascular liver diseases based on the best available evidence.

Abstract

Background

Hereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of colorectal and other cancers.

Methods

A team of 38 experts convened to update the 2008 European recommendations for the clinical management of patients with adenomatous polyposis syndromes. Additionally, other rare monogenic adenomatous polyposis syndromes were reviewed and added. Eighty-nine clinically relevant questions were answered after a systematic review of the existing literature with grading of the evidence according to Grading of Recommendations, Assessment, Development, and Evaluation methodology. Two levels of consensus were identified: consensus threshold (≥67% of voting guideline committee members voting either ‘Strongly agree’ or ‘Agree’ during the Delphi rounds) and high threshold (consensus ≥ 80%).

Results

One hundred and forty statements reached a high level of consensus concerning the management of hereditary adenomatous polyposis syndromes.

Conclusion

These updated guidelines provide current, comprehensive, and evidence-based practical recommendations for the management of surveillance and treatment of familial adenomatous polyposis patients, encompassing additionally MUTYH-associated polyposis, gastric adenocarcinoma and proximal polyposis of the stomach and other recently identified polyposis syndromes based on pathogenic variants in other genes than APC or MUTYH. Due to the rarity of these diseases, patients should be managed at specialized centres.