Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Anna Baiges, Fanny Turon, Macarena Simón-Talero, Stephanie Tasayco, Javier Bueno, Kamal Zekrini, Aurélie Plessier, Stéphanie Franchi-Abella, Florent Guerin, Amar Mukund, C. E. Eapen, Ashish Goel, Nidugala Keshava Shyamkumar, Sandra Coenen, Andrea De Gottardi, Avik Majumdar, Simona Onali, Akash Shukla, Flair José Carrilho, Lucas NacifMassimo Primignani, Giulia Tosetti, Vicenzo La Mura, Frederik Nevens, Peter Witters, Dhiraj Tripathi, Luis Tellez, Javier Martínez, Carmen Álvarez-Navascués, Miguel López Fraile López, Bogdan Procopet, Fabio Piscaglia, Barbara de Koning, Elba Llop, Mario Romero-Cristobal, Eric Tjwa, Alberto Monescillo-Francia, Marco Senzolo, Mercedes Perez-LaFuente, Antonio Segarra, Shiv Kumar Sarin, Virginia Hernández-Gea, David Patch, Wim Laleman, Hermien Hartog, Dominique Valla, Joan Genescà, Juan Carlos García-Pagán

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31 Citations (Scopus)


© 2019 by the American Association for the Study of Liver Diseases. 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.
Original languageEnglish
Publication statusPublished - 1 Jan 2019


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