TY - JOUR
T1 - Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study
AU - Baiges, Anna
AU - Turon, Fanny
AU - Simón-Talero, Macarena
AU - Tasayco, Stephanie
AU - Bueno, Javier
AU - Zekrini, Kamal
AU - Plessier, Aurélie
AU - Franchi-Abella, Stéphanie
AU - Guerin, Florent
AU - Mukund, Amar
AU - Eapen, C. E.
AU - Goel, Ashish
AU - Shyamkumar, Nidugala Keshava
AU - Coenen, Sandra
AU - De Gottardi, Andrea
AU - Majumdar, Avik
AU - Onali, Simona
AU - Shukla, Akash
AU - Carrilho, Flair José
AU - Nacif, Lucas
AU - Primignani, Massimo
AU - Tosetti, Giulia
AU - La Mura, Vicenzo
AU - Nevens, Frederik
AU - Witters, Peter
AU - Tripathi, Dhiraj
AU - Tellez, Luis
AU - Martínez, Javier
AU - Álvarez-Navascués, Carmen
AU - Fraile López, Miguel López
AU - Procopet, Bogdan
AU - Piscaglia, Fabio
AU - de Koning, Barbara
AU - Llop, Elba
AU - Romero-Cristobal, Mario
AU - Tjwa, Eric
AU - Monescillo-Francia, Alberto
AU - Senzolo, Marco
AU - Perez-LaFuente, Mercedes
AU - Segarra, Antonio
AU - Sarin, Shiv Kumar
AU - Hernández-Gea, Virginia
AU - Patch, David
AU - Laleman, Wim
AU - Hartog, Hermien
AU - Valla, Dominique
AU - Genescà, Joan
AU - García-Pagán, Juan Carlos
PY - 2019/1/1
Y1 - 2019/1/1
N2 - © 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.
AB - © 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.
U2 - 10.1002/hep.30817
DO - 10.1002/hep.30817
M3 - Article
C2 - 31211875
JO - Hepatology
JF - Hepatology
SN - 0270-9139
ER -