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

Hepatology - Tập 71 Số 2 - Trang 658-669 - 2020
Anna Baiges1, Fanny Turón1, Macarena Simón‐Talero2, Stephanie Tasayco2, Javier Bueno3, Kamal Zekrini4, Aurélie Plessier4, Stéphanie Franchi‐Abella5, Florent Guérin5, Amar Mukund6, C. E. Eapen7, Ashish Goel7, NK Shyamkumar8, Sandra Coenen9, Andrea De Gottardi10, Avik Majumdar11, Simona Onali11, Akash Shukla12, Flair José Carrilho13, Lucas Souto Nacif13, Massimo Primignani14, Giulia Tosetti14, Vincenzo La Mura14,15, Frederik Nevens16, Peter Witters16, Dhiraj Tripathi17, Luís Téllez18, Javier Martínez18, Montserrat Garcı́a-Retortillo19, Miguel Fraile‐López19, Bogdan Procopeţ20, Fabio Piscaglia21, Barbara de Koning22, Elba Llop23, M Ponce Romero24, Eric T.T.L. Tjwa25, Alberto Monescillo‐Francia26, Marco Senzolo27, Mercedes Pérez-Lafuente28, Antoni Segarra28, Shiv Kumar Sarin29, Jaume Bosch1, David Patch11, Wim Laleman15, Hermien Hartog9, Dominique Valla4, Joan Genescà2, Ángeles García‐Criado1
1Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd,Universitat de Barcelona,Barcelona,Spain
2Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, VHIR, CIBERehd,Universitat Autònoma de Barcelona,Barcelona,Spain
3Pediatric Surgery Department,Hospital Universitari Vall d'Hebrón,Universitat Autònoma de Barcelona,Barcelona,Spain
4DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie,Hôpital Beaujon, AP‐HP,Clichy,France
5Service d'Hépatologie et de Transplantation Hépatique et de radiologie Pédiatriques,Groupement Hospitalier Paris Sud (GHUPS), Hôpital Bicêtre,Le Kremlin Bicetre,France
6Department of Radiology, Institute of Liver and Biliary Sciences, New Delhi, India
7Hepatology Department, Christian Medical College, Vellore, India
8Department of Radiology, Christian Medical College, Vellore, India
9Department of Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands
10Hepatology, Clinic of Visceral Surgery and Medicine, Inselspital and Department of Clinical Research,University of Bern,Bern,Switzerland
11Sheila Sherlock Liver Unit and UCL Institute for Liver and Digestive Health,Royal Free Hospital and UCL,London,United Kingdom
12Department of Gastroenterology,Seth G S Medical College & KEM Hospital,Mumbai,Maharashtra,India
13Digestive Organ Transplantation Division, Department of Gastroenterology, Faculty of Medicine,University of São Paulo,São Paulo,Brazil
14A. M. e A. Migliavacca Center for Liver Disease, Division of Gastroenterology and Hepatology,Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan,Milan,Italy
15Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico,UOC Medicina Generale Emostasi e Trombosi,Milano,Italy
16Department of Gastroenterology and Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium
17Liver Unit,Queen Elisabeth Hospital,Birmingham,United Kingdom
18Department of Gastroenterology and Hepatology,Hospital Universitario Ramón y Cajal, IRYCIS,University of Alcalá,CIBERedh,Madrid,Spain
19Aparato Digestivo,Hospital Universitario Central de Asturias HUCA,Asturias,España
20Department of Gastroenterology, 3rd Medical Clinic,University of Medicine and Pharmacy “Iuliu Hatieganu”, Regional Institute of Gastroenterology and Hepatology “O Fodor”,Cluj‐Napoca,Romania
21Unit of Internal Medicine, Department of Medical and Surgical Sciences,University of Bologna, S. Orsola‐Malpighi Hospital,Bologna,Italy
22Division of Pediatric Gastroenterology,Erasmus MC‐Sophia Children's Hospital,Rotterdam,The Netherlands
23Liver Unit,Hospital U. Puerta de Hierro, Universidad Autónoma de Madrid,Madrid,Spain
24Digestive Diseases and Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas Hospital General Universitario Gregorio Marañón Instituto de Investigación Sanitaria Gregorio Marañón Facultad de Medicina,Universidad Complutense,Madrid,Spain
25Department of Gastroenterology and Hepatology, Radboud University Medical Centre, Nijmegen, The Netherlands
26Digestive Disease Department, Complejo Hospitalario Universitario Insular‐Materno Infantil,Las Palmas de Gran Canaria,España
27Multivisceral Transplant Unit, Department of Surgery, Oncology and Gastroenterology, Padua University Hospital, Padua, Italy
28Interventional Radiology Unit, Hospital Universitari Vall d'Hebrón,Universitat Autònoma de Barcelona,Barcelona,Spain
29Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India

Tóm tắt

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.

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Hepatology

Tập 71 Số 2

658-669

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