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

Lynch syndrome is the most common genetic predisposition for hereditary cancer but remains underdiagnosed. Large prospective observational studies have recently increased understanding of the effectiveness of colonoscopic surveillance and the heterogeneity of cancer risk between genotypes. The need for gene‐ and gender‐specific guidelines has been acknowledged.

Methods

The European Hereditary Tumour Group (EHTG) and European Society of Coloproctology (ESCP) developed a multidisciplinary working group consisting of surgeons, clinical and molecular geneticists, pathologists, epidemiologists, gastroenterologists, and patient representation to conduct a graded evidence review. The previous Mallorca guideline format was used to revise the clinical guidance. Consensus for the guidance statements was acquired by three Delphi voting rounds.

Results

Recommendations for clinical and molecular identification of Lynch syndrome, surgical and endoscopic management of Lynch syndrome‐associated colorectal cancer, and preventive measures for cancer were produced. The emphasis was on surgical and gastroenterological aspects of the cancer spectrum. Manchester consensus guidelines for gynaecological management were endorsed. Executive and layperson summaries were provided.

Conclusion

The recommendations from the EHTG and ESCP for identification of patients with Lynch syndrome, colorectal surveillance, surgical management of colorectal cancer, lifestyle and chemoprevention in Lynch syndrome that reached a consensus (at least 80 per cent) are presented.

Recommendations for clinical and molecular identification of LS, surgical and endoscopic management of LS‐associated colorectal cancer and preventive measures for cancer were produced. The emphasis was on surgical and gastroenterological aspects of the cancer spectrum.