Congenital veno‐venous malformations of the liver: Widely variable clinical presentations

Abstract

Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.

Diffuse Hepatic Metastasis—or Not?

“…They are divided in Abernethy (extrahepatic, types 1a, 1b, and 2) and Park (intrahepatic, types 1-4), and can be diagnosed incidentally, after liver tests changes, or by clinical signs of hepatic shunting (encephalopathy, hepatopulmonary syndrome, or hypoglycemia). 1 Congenital extrahepatic portosystemic shunt was first described by Abernethy in 1793, and later classified by Morgan as type 1, if the entire portal blood drains to the vena cava, and type 2 when there is a partial side-to-side anastomosis with a patent intrahepatic portal vein. To our knowledge, there are only 23 cases reported with the type 2 malformation.…”

Ductal paucity and Warkany syndrome in a patient with congenital extrahepatic portocaval shunt

“…Recurrent jaundice in CEPS has been described in the literature, but no explanation has been provided [7] . During embryogenesis, PV plays a crucial role in the formation and remodeling of the ductal plate.…”

Congenital hepatic shunt

“…At 8 weeks of age, unconjugated bilirubin levels were lower but still abnormal. At the time of this report, the patient was being monitored and had no clinical manifestations of the shunt.Congenital intrahepatic portosystemic shunts are rare and can be found incidentally in asymptomatic patients [1,2]. When present, symptoms can include hepatomegaly, metabolic disorders, encephalopathy, and jaundice [2].…”

“…Congenital intrahepatic portosystemic shunts are rare and can be found incidentally in asymptomatic patients [1,2]. When present, symptoms can include hepatomegaly, metabolic disorders, encephalopathy, and jaundice [2].…”

“…When present, symptoms can include hepatomegaly, metabolic disorders, encephalopathy, and jaundice [2]. CT and MRI can provide additional information, but color Doppler US is often the first diagnostic imaging test and may be sufficient [1].…”