Prenatal diagnosis and treatment of intrahepatic arteriovenous fistulas: case reports and the literature review

Abstract

Background: Congenital arteriovenous intrahepatic fistulas, which are hepatic hemangiomas and arteriovenous malformations (AVMs) are rare and they confused with each other. Knowledge of prenatal medical treatment of AVMs is insufficient.

Objectives: First is to emphasize the distinction between hepatic hemangioma and AVMs. Second is discussion of the first case of hepatic AVM that responded well to steroid-propranolol treatment in the prenatal period.

Methods: Color Doppler ultrasonography, fetal and postnatal MR were used for diagnosis.

Results: The first case is a giant hepatic hemangioma diagnosed and progressively growing in the prenatal period and gradually shrinking in the postnatal period. The second case was hepatic AVM with no signs of heart failure during the prenatal period and postnatal right extended hepatectomy was performed as the anastomosis was enlarged and intraportal collateral vessels were developed. The third case is the first hepatic AVM which reaches a term that was prenatally diagnosed and responded to treatment with marked reduction.

Conclusion: Color flow and pulse Doppler imaging have a key role in the prenatal diagnosis of arteriovenous fistulas. Intrahepatic AVM are abnormal intrahepatic vascular network formation primarily fed by the hepatic artery or its branches and drained by the hepatic venous system. This vascular region looks like a mass, but it does not contain a solid area, which allows the separation of hepatic AVMs from hepatic hemangiomas. Steroid and propranolol therapy should be considered in management.

Keywords: Arteriovenous malformation; Doppler ultrasound; hepatic hemangioma; intrahepatic arteriovenous fistula; steroid and propranolol treatment.