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 due to 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.
Congenital extrahepatic portosystemic shunts (Abernethy malformation) : an international observational study / A. Baiges, F. Turon, M. Simón-Talero, S. Tasayco, J. Bueno, K. Zekrini, A. Plessier, S. Franchi-Abella, F. Guerin, A. Mukund, C.E. Eapen, A. Goel, N.K. Shyamkumar, S. Coenen, A. De Gottardi, A. Majumdar, S. Onali, A. Shukla, J. Carrilho Flair, L. Nacif, M. Primignani, G. Tosetti, V. La Mura, F. Nevens, P. Witters, D. Tripathi, L. Tellez, J. Martínez, C. Álvarez-Navascués, M. Fraile, B. Procopet, F. Piscaglia, B. de Koning, E. Llop, M. Romero Cristobal, E. Tjwa, A. Monescillo-Francia, M. Senzolo, M. Perez-LaFuente, A. Segarra, K. Sarin Shiv, V. Hernández-Gea, D. Patch, W. Laleman, H. Hartog, D. Valla, J. Genescà, C. García-Pagán Joan. - In: HEPATOLOGY. - ISSN 0270-9139. - (2019 Jun 18). [Epub ahead of print] [10.1002/hep.30817]
Congenital extrahepatic portosystemic shunts (Abernethy malformation) : an international observational study
G. Tosetti;V. La Mura;
2019
Abstract
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 due to 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.
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