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Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study.
Baiges, Anna; Turon, Fanny; Simón-Talero, Macarena; Tasayco, Stephanie; Bueno, Javier; Zekrini, Kamal; Plessier, Aurélie; Franchi-Abella, Stéphanie; Guerin, Florent; Mukund, Amar; Eapen, C E; Goel, Ashish; Shyamkumar, Nidugala Keshava; Coenen, Sandra; De Gottardi, Andrea; Majumdar, Avik; Onali, Simona; Shukla, Akash; Carrilho, Flair José; Nacif, Lucas; Primignani, Massimo; Tosetti, Giulia; La Mura, Vicenzo; Nevens, Frederik; Witters, Peter; Tripathi, Dhiraj; Tellez, Luis; Martínez, Javier; Álvarez-Navascués, Carmen; Fraile López, Miguel López; Procopet, Bogdan; Piscaglia, Fabio; de Koning, Barbara; Llop, Elba; Romero-Cristobal, Mario; Tjwa, Eric; Monescillo-Francia, Alberto; Senzolo, Marco; Perez-LaFuente, Mercedes; Segarra, Antonio; Sarin, Shiv Kumar; Hernández-Gea, Virginia; Patch, David; Laleman, Wim; Hartog, Hermien; Valla, Dominique; Genescà, Joan; García-Pagán, Juan Carlos.
Afiliación
  • Baiges A; Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, Universitat de Barcelona, Barcelona, Spain.
  • Turon F; Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, Universitat de Barcelona, Barcelona, Spain.
  • Simón-Talero M; Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, VHIR, CIBERehd, Universitat Autònoma de Barcelona, Barcelona, Spain.
  • Tasayco S; Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, VHIR, CIBERehd, Universitat Autònoma de Barcelona, Barcelona, Spain.
  • Bueno J; Pediatric Surgery Department, Hospital Universitari Vall d'Hebrón, Universitat Autònoma de Barcelona, Barcelona, Spain.
  • Zekrini K; DHU 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.
  • Plessier A; DHU 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.
  • Franchi-Abella S; Service d'Hépatologie et de Transplantation Hépatique et de radiologie Pédiatriques, Groupement Hospitalier Paris Sud (GHUPS), Hôpital Bicêtre, Le Kremlin Bicetre, France.
  • Guerin F; Service d'Hépatologie et de Transplantation Hépatique et de radiologie Pédiatriques, Groupement Hospitalier Paris Sud (GHUPS), Hôpital Bicêtre, Le Kremlin Bicetre, France.
  • Mukund A; Department of Radiology, Institute of Liver and Biliary Sciences, New Delhi, India.
  • Eapen CE; Hepatology Department, Christian Medical College, Vellore, India.
  • Goel A; Hepatology Department, Christian Medical College, Vellore, India.
  • Shyamkumar NK; Department of Radiology, Christian Medical College, Vellore, India.
  • Coenen S; Department of Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands.
  • De Gottardi A; Hepatology, Clinic of Visceral Surgery and Medicine, Inselspital and Department of Clinical Research, University of Bern, Bern, Switzerland.
  • Majumdar A; Sheila Sherlock Liver Unit and UCL Institute for Liver and Digestive Health, Royal Free Hospital and UCL, London, United Kingdom.
  • Onali S; Sheila Sherlock Liver Unit and UCL Institute for Liver and Digestive Health, Royal Free Hospital and UCL, London, United Kingdom.
  • Shukla A; Department of Gastroenterology, Seth G S Medical College & KEM Hospital, Mumbai, Maharashtra, India.
  • Carrilho FJ; Digestive Organ Transplantation Division, Department of Gastroenterology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.
  • Nacif L; Digestive Organ Transplantation Division, Department of Gastroenterology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.
  • Primignani M; A. 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.
  • Tosetti G; A. 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.
  • La Mura V; A. 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.
  • Nevens F; Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, UOC Medicina Generale Emostasi e Trombosi, Milano, Italy.
  • Witters P; Department of Gastroenterology and Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium.
  • Tripathi D; Department of Gastroenterology and Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium.
  • Tellez L; Liver Unit, Queen Elisabeth Hospital, Birmingham, United Kingdom.
  • Martínez J; Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, IRYCIS, University of Alcalá, CIBERedh, Madrid, Spain.
  • Álvarez-Navascués C; Department of Gastroenterology and Hepatology, Hospital Universitario Ramón y Cajal, IRYCIS, University of Alcalá, CIBERedh, Madrid, Spain.
  • Fraile López ML; Aparato Digestivo, Hospital Universitario Central de Asturias HUCA, Asturias, España.
  • Procopet B; Aparato Digestivo, Hospital Universitario Central de Asturias HUCA, Asturias, España.
  • Piscaglia F; Department of Gastroenterology, 3rd Medical Clinic, University of Medicine and Pharmacy "Iuliu Hatieganu", Regional Institute of Gastroenterology and Hepatology "O Fodor", Cluj-Napoca, Romania.
  • de Koning B; Unit of Internal Medicine, Department of Medical and Surgical Sciences, University of Bologna, S. Orsola-Malpighi Hospital, Bologna, Italy.
  • Llop E; Division of Pediatric Gastroenterology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
  • Romero-Cristobal M; Liver Unit, Hospital U. Puerta de Hierro, Universidad Autónoma de Madrid, Madrid, Spain.
  • Tjwa E; Digestive 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.
  • Monescillo-Francia A; Department of Gastroenterology and Hepatology, Radboud University Medical Centre, Nijmegen, The Netherlands.
  • Senzolo M; Digestive Disease Department, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, España.
  • Perez-LaFuente M; Multivisceral Transplant Unit, Department of Surgery, Oncology and Gastroenterology, Padua University Hospital, Padua, Italy.
  • Segarra A; Interventional Radiology Unit, Hospital Universitari Vall d'Hebrón, Universitat Autònoma de Barcelona, Barcelona, Spain.
  • Sarin SK; Interventional Radiology Unit, Hospital Universitari Vall d'Hebrón, Universitat Autònoma de Barcelona, Barcelona, Spain.
  • Hernández-Gea V; Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
  • Patch D; Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, Universitat de Barcelona, Barcelona, Spain.
  • Laleman W; Sheila Sherlock Liver Unit and UCL Institute for Liver and Digestive Health, Royal Free Hospital and UCL, London, United Kingdom.
  • Hartog H; Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, UOC Medicina Generale Emostasi e Trombosi, Milano, Italy.
  • Valla D; Department of Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands.
  • Genescà J; DHU 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.
  • García-Pagán JC; Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, VHIR, CIBERehd, Universitat Autònoma de Barcelona, Barcelona, Spain.
Hepatology ; 71(2): 658-669, 2020 02.
Article en En | MEDLINE | ID: mdl-31211875
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Vena Porta / Encefalopatía Hepática / Síndrome Hepatopulmonar / Malformaciones Vasculares / Hipertensión Pulmonar / Neoplasias Hepáticas Tipo de estudio: Diagnostic_studies / Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Aged / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged Idioma: En Revista: Hepatology Año: 2020 Tipo del documento: Article País de afiliación: España Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Vena Porta / Encefalopatía Hepática / Síndrome Hepatopulmonar / Malformaciones Vasculares / Hipertensión Pulmonar / Neoplasias Hepáticas Tipo de estudio: Diagnostic_studies / Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Aged / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged Idioma: En Revista: Hepatology Año: 2020 Tipo del documento: Article País de afiliación: España Pais de publicación: Estados Unidos