Congenital absence of the portal vein in an adult woman: A case report

Congenital absence of the portal vein is a rare malformation. Mesenteric and splenic venous blood bypasses the liver and drains into the renal veins or directly into the inferior vena cava. The malformation occurs predominantly in females and children and is often associated with other anomalies such as hepatic tumors, cardiac malformations and skeletal abnormalities. We describe a 23-year-old female with congenital absence of the portal vein associated with variant origin of the hepatic artery from the superior mesenteric artery. Additional anomalies were not present. The patient is in good condition. Laboratory tests showed elevated total bilirubin; other liver parameters were within normal range. The absence of the portal vein was confirmed by MR angiography, which revealed that the upper part of the superior mesenteric vein and the splenic vein drained into the left renal vein. A liver biopsy showed no signs of cirrhosis. Yearly follow-up with laboratory tests and ultrasonography was recommended to our patient. According to the literature, the prognosis of this patient depends on the presence of cardiac defects and liver tumors. There is little information on the outcome of patients with congenital absence of the portal vein; only long-term follow-up investigations will provide answers to the numerous remaining questions.