Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

CE detects more lesions than CTA or standard mesenteric angiography in patients with obscure GI bleeding and has a therapeutic impact in almost half of the patients with positive findings.

The liver has a unique dual blood supply, which makes helical computed tomography (CT) a highly suitable technique for hepatic imaging. Helical CT allows single breath-hold scanning without motion artifacts. Because of rapid image acquisition, two-phase (hepatic arterial phase and portal venous phase) evaluation of the hepatic parenchyma is possible, improving tumor detection and tumor characterization in a single CT study. The arterial and portal venous supplies to the liver are not independent systems. There are several communications between the vessels, including transsinusoidal, transvasal, and transplexal routes. When vascular compromise occurs, there are often changes in the volume of blood flow in individual vessels and even in the direction of blood flow. These perfusion disorders can be detected with helical CT and are generally seen as an area of high attenuation on hepatic arterial phase images that returns to normal on portal venous phase images; this finding reflects increased arterial blood flow and arterioportal shunting in most cases. Familiarity with the helical CT appearances of these perfusion disorders will result in more accurate diagnosis. By recognizing these perfusion disorders, false-positive diagnosis (hypervascular tumors) or overestimation of the size of liver tumors (eg, hepatocellular carcinoma) can be avoided.

Hemorrhage after pancreatic resection must be considered a complication with relatively high mortality. Diagnosis should be established and treatment applied rapidly. Pancreatic and/or biliary fistulae were significantly associated with a higher risk of postoperative hemorrhage. Interventional radiology is a good therapeutic option.

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