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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.

Gastroesophageal variceal bleeding in patients with cirrhosis is associated with significant morbidity and mortality, as well as a high rebleeding risk. Limited data are available on the role of transjugular intrahepatic portosystemic shunt (TIPS) with covered stents in patients receiving standard endoscopic, vasoactive, and antibiotic treatment. In this multicenter randomized trial, long-term endoscopic variceal ligation (EVL) or glue injection 1 b-blocker treatment was compared with TIPS placement in 72 patients with a first or second episode of gastric and/or esophageal variceal bleeding, after hemodynamic stabilization upon endoscopic, vasoactive, and antibiotic treatment. Randomization was stratified according to Child-Pugh score. Kaplan-Meier (event-free) survival estimates were used for the endpoints rebleeding, death, treatment failure, and hepatic encephalopathy. During a median follow-up of 23 months, 10 (29%) of 35 patients in the endoscopy 1 b-blocker group, as compared to 0 of 37 (0%) patients in the TIPS group, developed variceal rebleeding (P 5 0.001). Mortality (TIPS 32% vs. endoscopy 26%; P 5 0.418) and treatment failure (TIPS 38% vs. endoscopy 34%; P 5 0.685) did not differ between groups. Early hepatic encephalopathy (within 1 year) was significantly more frequent in the TIPS group (35% vs. 14%; P 5 0.035), but during longterm follow-up this difference diminished (38% vs. 23%; P 5 0.121). Conclusions: In unselected patients with cirrhosis, who underwent successful endoscopic hemostasis for variceal bleeding, covered TIPS was superior to EVL 1 b-blocker for reduction of variceal rebleeding, but did not improve survival. TIPS was associated with higher rates of early hepatic encephalopathy. (HEPATOLOGY 2016;63:581-589) G astroesophageal variceal bleeding (GEVB) is a severe complication of portal hypertension. Rebleeding is associated with significant morbidity and mortality. Hospitalization costs for rebleeding range between $6,600 and $23,000 in the United States.For these reasons, management should be directed at its prevention.1,2 Secondary prevention is first achieved by endoscopic treatment (endoscopic variceal ligation [EVL] for esophageal varices and N-butyl cyanoacrylate injection for gastric varices) in combination with bAbbreviations: CI, confidence interval; EVL, endoscopic variceal ligation; GEVB, gastroesophageal variceal bleeding; HCC, hepatocellular carcinoma; HR, hazard ratio; IQR, interquartile range; MELD, Model for End-Stage Liver Disease; PTFE, polytetrafluorethylene; TIPS, transjugular intrahepatic portosystemic shunt.From the Departments of