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.
Looze et al. (Thu,) studied this question.