Congenital symptomatic intrahepatic arteriovenous fistulas in newborns: management of 2 cases with prenatal diagnosis

Abstract

Background: Fetal and neonatal hepatic arteriovenous fistulas are rare and associated with a high mortality rate; they can be prenatally detected by ultrasonography. Management of these malformations can be a challenge for pediatric surgeons.

Methods: Two patients with a prenatal diagnosis of intrahepatic arteriovenous shunts were treated at our institution in the last 2 years. A hepatic complex arteriovenous malformation fed respectively by prominent branches of the hepatic artery and of the celiac trunk rising from dilated suprarenal aortae and draining into suprahepatic veins was detected. In the first case, an embolization was performed; in the second, the surgical resection of the vascular malformation was the treatment of choice.

Results: The first patient died after embolization and before surgery for hemodynamic complications. The second patient, at a follow-up of 16 months, is alive and doing well.

Conclusion: Hepatic resection is the treatment of choice for localized intrahepatic arteriovenous malformation. Theoretically, embolization could be curative or reduce the size of a malformation, making consequent hepatic resection feasible. Results do not support this theory because of the high rate of complications recorded that brought in every case, ours included, to the death of the child.