Pancreatic Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Comprehensive Review of Existing Cases
By
Isabel de Jager
Ebba Asplund
Nikolaos Kartalis
Poya Ghorbani
Nina Bloch
Johannes Matthias Löhr
Miroslav Vujasinovic
September 25, 2026
Clinical Scorecard: Pancreatic Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Comprehensive Review of Existing Cases
At a Glance
Category Detail
Condition Pancreatic Arteriovenous Malformations (AVMs) in Hereditary Hemorrhagic Telangiectasia (HHT)
Key Mechanisms Loss of regulatory sphincteric control at the arteriolar–capillary junction leading to unregulated arterial flow and arteriovenous shunts.
Target Population Individuals with Hereditary Hemorrhagic Telangiectasia (HHT)
Care Setting Clinical evaluation and management of vascular malformations
Key Highlights
19 patients with pancreatic AVMs in HHT identified across 13 studies. Mean AVM size in HHT patients was 7.1 mm, significantly smaller than general pancreatic AVMs. 50% of patients were asymptomatic; 73% had no laboratory abnormalities. Conservative management was used in 69% of cases. Serious complications occurred less frequently in HHT patients.
Guideline-Based Recommendations
Diagnosis
Diagnosis based on the Curaçao criteria, requiring at least three criteria to be fulfilled.
Management
Conservative management and surveillance for asymptomatic cases are recommended.
Monitoring & Follow-up
Further research is needed to clarify prognosis and treatment outcomes.
Risks
Potential complications include portal hypertension and gastrointestinal bleeding.
Patient & Prescribing Data
Patients with Hereditary Hemorrhagic Telangiectasia (HHT) presenting with pancreatic AVMs.
Surgical intervention is generally reserved for symptomatic patients due to potential complications.
Clinical Best Practices
Consider conservative management for asymptomatic pancreatic AVMs in HHT. Monitor for complications such as portal hypertension and gastrointestinal bleeding.
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