Hepatic arterio-portal-venous malformation in children: the diagnosis and interventional treatment

Objective: To study the diagnosis and interventional treatment of arterio-portal-venous malformation (APVM) in liver. Methods: 2 patients were male and 2 patients were female. The age was 3.5, 4, 12, 13 years old respectively. Clinical manifestations included repeated upper gastrointestinal hemorrhage and refractory anemia were misdiagnosed as hepatocirrhosis. After confirmed by hepatic arteriography, APVM were embolized with gelfoam plus spring coils (n=2) or polyvinyl alcohol (PVA) particles (n=2) respectively. Results: All patients hepatic function and alpha-fetoprotein level were normal. CT scan and Doppler ultrasound showed normal or slightly enlarged hepatic volume and no space- occupying lesion with signs of portal hypertension. Arteriography revealed enlarged and numerous branches of hepatic artery with abnormal portal vein visualization and varicose coronary vein. Gastrointestinal hemorrhage was stopped in three children and recurred in another after first embolization. One boy had survived for 24 months up to now, another died of uremia after 13 months and others died 15 days, 3 months after embolization respectively. Conclusion: APVM is a congenital hepatic disease and easily misdiagnosed by podiatrist. Arteriography is the golden standard of diagnosis. Trans-arterial embolization of APVM is the main therapeutic method. Materials of embolization included gelfoam, spring coil and PVA particles, of which the latter is better. (authors)