Complex Management of High-Flow Gastrointestinal Arteriovenous Malformation With Chronic Portomesenteric Thrombosis in PTEN Hamartoma Tumor Syndrome

ACG Case Reports Journal 2026 Open access

Read full text
Authors: Katrien De Looze, Kymentie Ferdinande, Laurence Levrau, Uri Singfer, Luis De Carvalho, Anna Vantilborgh, Margot Callens, Aude Beyens, Anne Hoorens, Anja M. Geerts, Xavier Verhelst, Sarah Raevens
+1 more Hans Van Vlierberghe
DOI: 10.14309/crj.0000000000001942
Cited by: 0
References: 16

Abstract

We present a rare case of life-threatening gastrointestinal bleeding from a high-flow terminal ileal arteriovenous malformation (AVM) in a 19-year-old woman, complicated by chronic portomesenteric thrombosis. Management included sequential targeted embolizations, anticoagulation and venous stenting in attempt to recanalize the portomesenteric vasculature to lower the pressure in the AVM, and, ultimately, right hemicolectomy with portal recanalization. Genetic analysis confirmed phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome, suggesting a potential link between PTEN hamartoma tumor syndrome and visceral vascular anomalies. This case underscores the challenges of treating complex AVMs in the setting of thrombosis and highlights the importance of multidisciplinary, individualized management, with international expert consultation essential in rare vascular presentations.

Fields of Study & Subjects

Vascular Anomalies and Treatments Gastrointestinal Bleeding Diagnosis and Treatment Vascular Malformations and Hemangiomas

Publication details

Journal / source
ACG Case Reports Journal
Publisher
Ovid Technologies (Wolters Kluwer Health)
Volume
13
Issue
1
Pages
e01942–e01942
Publication date
1 January 2026
Language
EN