JHEP Reports (Jan 2024)
Expert management of congenital portosystemic shunts and their complicationsKey points
- Valérie Anne McLin,
- Stéphanie Franchi-Abella,
- Timothée Brütsch,
- Atessa Bahadori,
- Valeria Casotti,
- Jean de Ville de Goyet,
- Grégoire Dumery,
- Emmanuel Gonzales,
- Florent Guérin,
- Sebastien Hascoet,
- Nigel Heaton,
- Béatrice Kuhlmann,
- Frédéric Lador,
- Virginie Lambert,
- Paolo Marra,
- Aurélie Plessier,
- Alberto Quaglia,
- Anne-Laure Rougemont,
- Laurent Savale,
- Moinak Sen Sarma,
- Olivier Sitbon,
- Riccardo Antonio Superina,
- Hajime Uchida,
- Mirjam van Albada,
- Hubert Petrus Johannes van der Doef,
- Valérie Vilgrain,
- Julie Wacker,
- Nitash Zwaveling,
- Dominique Debray,
- Barbara Elisabeth Wildhaber
Affiliations
- Valérie Anne McLin
- Swiss Pediatric Liver Center, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland; ERN RARE LIVER; Corresponding author. Address: Swiss Pediatric Liver Center, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
- Stéphanie Franchi-Abella
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Centre de référence des maladies rares du foie de l’enfant, Service de radiologie pédiatrique diagnostique et interventionnelle, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; BIOMAPS UMR 9011 CNRS, INSERM, CEA, Orsay, France; ERN RARE LIVER; ERN Transplant Child
- Timothée Brütsch
- Communication in Science, rue du Tunnel 7, Carouge, Switzerland
- Atessa Bahadori
- Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
- Valeria Casotti
- Pediatric Hepatology, Gastroenterology and Transplant Centre, ASST Papa Giovanni XXIII Hospital, Bergamo, Italy; ERN Transplant Child
- Jean de Ville de Goyet
- Pediatric Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, ISMETT UPMC, Palermo, Italy
- Grégoire Dumery
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Service de gynécologie et d’obstétrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
- Emmanuel Gonzales
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Centre de référence des maladies rares du foie de l’enfant, FHU Hepatinov, Service d’hépatologie et transplantation hépatique pédiatriques, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMRS_1193, Orsay, France; ERN RARE LIVER; ERN Transplant Child
- Florent Guérin
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Service de chirurgie pédiatrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; ERN RARE LIVER; ERN Transplant Child
- Sebastien Hascoet
- Department of Congenital Heart Diseases, Hôpital Marie Lannelongue, France; INSERM UMR_S 999, Université Paris, France
- Nigel Heaton
- Institute of Liver Studies, Kings College Hospital, London, England
- Béatrice Kuhlmann
- Pediatric Endocrinology, Cantonal Hospital Aarau KSA, Aarau, Switzerland
- Frédéric Lador
- Service de Pneumologie, University of Geneva, Geneva, Switzerland
- Virginie Lambert
- Cardiologie congénitale, Institut Mutualiste Montsouris, Paris, France; AP-HP, Centre de référence des maladies rares du foie de l’enfant, Service de radiologie pédiatrique diagnostique et interventionnelle, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
- Paolo Marra
- Department of Radiology, Papa Giovanni XXIII Hospital, School of Medicine and Surgery - University of Milano-Bicocca, Bergamo, Italy
- Aurélie Plessier
- Centre de référence des maladies vasculaires du foie, Service d’hépatologie Hôpital Beaujon, Clichy, France; ERN RARE LIVER; VALDIG
- Alberto Quaglia
- Department of Cellular Pathology, Royal Free London NHS Foundation Trust/UCL Cancer Institute, London, England
- Anne-Laure Rougemont
- Swiss Pediatric Liver Center, Division of Clinical Pathology, Diagnostic Department, University of Geneva, Geneva, Switzerland
- Laurent Savale
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Centre de référence de l’hypertension pulmonaire, Service de pneumologie et soins intensifs respiratoires, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France; ERN Lung
- Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
- Olivier Sitbon
- Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France; AP-HP, Centre de référence de l’hypertension pulmonaire, Service de pneumologie et soins intensifs respiratoires, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France; ERN Lung
- Riccardo Antonio Superina
- Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, USA
- Hajime Uchida
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
- Mirjam van Albada
- Department of paediatric and congenital cardiology, University Medical Center Groningen, University of Groningen, The Netherlands
- Hubert Petrus Johannes van der Doef
- Division of paediatric gastroenterology and hepatology, Department of paediatrics, University Medical Center Groningen, Groningen, The Netherlands
- Valérie Vilgrain
- Université Paris Cité, CRI, INSERM, Paris, France; AP-HP, Département de Radiologie, Hôpital Beaujon. Nord, Clichy, France; ERN RARE LIVER; VALDIG
- Julie Wacker
- Pediatric Cardiology Unit, Department of pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland; Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Geneva and Lausanne, Switzerland
- Nitash Zwaveling
- Department of Pediatric Endocrinology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands
- Dominique Debray
- AP-HP, Unité d’hépatologie pédiatrique et transplantation hépatique, Hôpital Necker, Paris, France; Centre de Référence des maladies rares du foie de l’enfant, FILFOIE, France; ERN RARE LIVER; ERN Transplant Child
- Barbara Elisabeth Wildhaber
- Swiss pediatric Liver Center, Division of pediatric surgery, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland; ERN RARE LIVER
- Journal volume & issue
-
Vol. 6,
no. 1
p. 100933
Abstract
Summary: Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.
