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

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.