Expert management of congenital portosystemic shunts and their complications

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 SarmaOlivier 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

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations

Abstract

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.

Original languageEnglish (US)
Article number100933
JournalJHEP Reports
Volume6
Issue number1
DOIs
StatePublished - Jan 2024

Funding

The authors are grateful to the following bodies for their support of the symposium: the European Joint Program for Rare Disease Networking Support Scheme (EJP RD NSS), the Swiss Association for the Study of the Liver (SASL), and the European Society for Pediatric Gastroenterology and Nutrition (ESPGHAN), ERN RARE LIVER and the VALDIG group. Thanks also go out to the University of Geneva, the University Hospitals Geneva Communications Department, and Drs Barooty, Salamoni and Steger for logistical support. Finally, the authors are indebted to Cristina Späni and Jérôme Divorne for their invaluable contributions to the meeting of experts and to Simona Korff for her management of the IRCPSS. The Registry is supported by ESPGHAN and the European Association for the Study of the Liver (EASL). The Meeting of Experts was funded by EJP RD NSS, SASL, ESPGHAN. The registry is sponsored by a registry grant from EASL and a network grant from ESPGHAN. The authors are grateful to the following bodies for their support of the symposium: the European Joint Program for Rare Disease Networking Support Scheme (EJP RD NSS), the Swiss Association for the Study of the Liver (SASL), and the European Society for Pediatric Gastroenterology and Nutrition (ESPGHAN), ERN RARE LIVER and the VALDIG group. Thanks also go out to the University of Geneva, the University Hospitals Geneva Communications Department, and Drs Barooty, Salamoni and Steger for logistical support. Finally, the authors are indebted to Cristina Späni and Jérôme Divorne for their invaluable contributions to the meeting of experts and to Simona Korff for her management of the IRCPSS. The Registry is supported by ESPGHAN and the European Association for the Study of the Liver (EASL). In addition, the authors are indebted to the following participants for their valuable clinical contributions and discussions: Ana Baiges, Hepatology, Hospital Clínic de Barcelona, Barcelona, Spain; Ron Bardin, Helen Schneider's Hospital for Women, Rabin Medical Center, Petach Tikva, Israel; Audrey Baron, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Centre, Hôpital Bicêtre, Le Kremlin Bicêtre, Paris, France; Ana Calinescu, Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland; Vittoria Camelli, Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, AOU Città della Salute e della Scienza di Torino, Italy; Debalina Chakrabarti, Dept of Radiodiagnosis, Avadh Hospital and Heart Center, Lucknow 226005, India; Nicolas Foureur, Centre d’éthique clinique de l'AP-HP, Hôpital Cochin, Paris, France; Mehdi Gadiri, University of Geneva, Geneva, Switzerland; Solène Le Cam, 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; Caroline Lemoine, Ann and Robert Lurie Children's Hospital of Chicago, Chicago, USA; Josefina Martinelli, Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland; Periklis Makrythanasis, Medical School, National and Kapodistrian University of Athens, Athens, Greece; Jai Patel, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom; Tudor Pop, 2nd Pediatric Clinic of the University of Medicine and Pharmacy Cluj-Napoca, Romania; Alexis Ricoeur, Unité de radiologie abdominale et interventionnelle, Département diagnostic, University Hospitals of Geneva, Geneva, Switzerland; Fabien Robert, INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis Robinson, France; Nathalie Rock, Swiss Pediatric Liver Center, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland; Vanessa Steger, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland; Frank van Steenbeek, University of Utrecht, Utrecht, Netherlands.

Keywords

  • adenoma
  • congenital portosystemic shunt
  • focal nodular hyperplasia
  • hepatocellular carcinoma
  • hyperandrogenism
  • hyperinsulinism
  • hypoglycemia
  • occlusion test
  • portal pressure
  • puberty
  • pulmonary hypertension
  • β-catenin

ASJC Scopus subject areas

  • Internal Medicine
  • Immunology and Allergy
  • Hepatology
  • Gastroenterology

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