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.

Safety of regorafenib in hepatocellular carcinoma (HCC) recurrence after liver transplantation (LT) has been recently demonstrated. We aimed to assess the survival benefit of regorafenib compared with best supportive care (BSC) in LT patients after sorafenib discontinuation. This observational multicenter retrospective study included LT patients with HCC recurrence who discontinued first‐line sorafenib. Group 1 comprised regorafenib‐treated patients, whereas the control group was selected among patients treated with BSC due to unavailability of second‐line options at the time of sorafenib discontinuation and who were sorafenib‐tolerant progressors (group 2). Primary endpoint was overall survival (OS) of group 1 compared with group 2. Secondary endpoints were safety and OS of sequential treatment with sorafenib + regorafenib/BSC. Among 132 LT patients who discontinued sorafenib included in the study, 81 were sorafenib tolerant: 36 received regorafenib (group 1) and 45 (group 2) received BSC. Overall, 24 (67%) patients died in group 1 and 40 (89%) in group 2: the median OS was significantly longer in group 1 than in group 2 (13.1 versus 5.5 months; P < 0.01). Regorafenib treatment was an independent predictor of reduced mortality (hazard ratio, 0.37; 95% confidence interval [CI], 0.16‐0.89; P = 0.02). Median treatment duration with regorafenib was 7.0 (95% CI, 5.5‐8.5) months; regorafenib dose was reduced in 22 (61%) patients for adverse events and discontinued for tumor progression in 93% (n = 28). The median OS calculated from sorafenib start was 28.8 months (95% CI, 17.6‐40.1) in group 1 versus 15.3 months (95% CI, 8.8‐21.7) in group 2 (P < 0.01). Regorafenib is an effective second‐line treatment after sorafenib in patients with HCC recurrence after LT.

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Double-tunnel circumferential endoscopic submucosal dissection with double clipband-line traction for an esophageal squamous neoplasm ▶ Fig. 1 Schematic of the steps involved in the double-tunnel and double-traction method for resection of a large superficial esophageal lesion including: a a circumferential anal incision; b incision and creation of the first tunnel; c, d the second incision and tunnel created at the opposite end of the circumference; e the "clip band line" placed on the first pillar; f dissection of the first pillar; g the second "clip band line" on the second pillar and dissection of the second pillar; h the completed circumferential endoscopic submucosal dissection. Fraile-López Miguel, Parra-Blanco Adolfo. Double-tunnel, double-traction ESD with the clip-band-line method … Endoscopy UK Video 1 Double-tunnel and double-traction method applied on both pillars for circumferential esophageal endoscopic submucosal dissection. ▶ Fig. 2 Endoscopic views during the double-tunnel and double-traction endoscopic submucosal dissection showing: a the esophageal lesion with Lugol stain; b the circumferential anal cut; c the first tunnel; d the second tunnel; e dissection of the first pillar with the use of clip-band-line traction; f dissection of the second pillar with a second "clip band line" applied; g the circumferential scar. h Macroscopic appearance of the resected specimen. Fraile-López Miguel, Parra-Blanco Adolfo. Double-tunnel, double-traction ESD with the clip-band-line method … Endoscopy E-VideosThis document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

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