Abstract / Summary
Objectives: Limited data are available on pancreatic arteriovenous malformations (AVMs) in patients with hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease. This systematic review aimed to identify all reported cases of pancreatic AVMs in patients with HHT and summarise patient demographics, clinical presentation, diagnostic modalities, treatment strategies and follow-up.
Design: Systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
Data sources: Databases PubMed, Embase, Cochrane Library and Web of Science were systematically screened up to June 2025.
Eligibility criteria: All English-language, full-text studies reporting pancreatic AVMs in patients with HHT. Only patients with definite diagnoses of HHT and pancreatic AVM were included.
Data extraction and synthesis: All identified articles were independently screened by two reviewers. Data regarding patient demographics, clinical presentation, diagnostic modalities, treatment strategies and follow-up were collected. The mean AVM size was calculated when individual AVM size and number of AVMs were reported.
Results: Of 429 screened records, 46 articles underwent full-text assessment. Nineteen patients from 13 studies met the inclusion criteria. Most patients were female (58%), with a mean age of 52 years, presenting asymptomatically (50%) without laboratory findings (73%). CT was the most frequently used diagnostic modality (56%), and the mean AVM size was 7.1 mm. Most patients were treated conservatively (69%). Prognosis was frequently unavailable (79%). Differential diagnoses consisted mostly of neuroendocrine tumours (75%).
Conclusions: Pancreatic AVMs in patients with HHT appear to be smaller and are more frequently asymptomatic compared with the general population. Severe complications, such as portal hypertension and gastrointestinal bleeding, were less frequent. Conservative management was most common. Additional studies are needed to better characterise prognosis and treatment outcomes, particularly in symptomatic patients.
Prospero registration number: CRD420251077109.