Pulsatile hepatofugal flow in the portal vein: hallmark of a congenital hepatoportal arteriovenous fistula.

After an uneventful pregnancy and perinatal period, the second child of healthy nonconsanguineous parents was admitted at the age of 6 weeks because of failure to thrive. On physical examination, he had massive ascites and splenomegaly. He had mild normochrome anemia (hemoglobin, 5.4 mmol/L; normal, >5.9 mmol/L); blood smear and platelet count were normal, as were liver function tests. Screening for metabolic disorders was negative. The serum/ascites ratio was 0.16 both for total protein and lactate dehydrogenase, classifying the ascites as a transudate. Broad coagulation studies failed to demonstrate any signs of thrombophilia. During diagnostic workup, the boy developed clinical and radiological signs of necrotizing enterocolitis with positive blood cultures for Escherichia coli. Only then a marked thickening of his bowel walls with intramural air and intrasplenic air inclusions was noted on ultrasound. Duplex Doppler investigation showed