Congenital Portosystemic Shunts
Current Diagnosis and Management
Corresponding Author
Valérie A. McLin
Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland
Address correspondence and reprint requests to Valérie A. McLin, MD, Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland (e-mail: [email protected]).Search for more papers by this authorStephanie Franchi Abella
Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, University Paris-Sud, Orsay
Pediatric Liver Transplantation Unit, Reference Centre for Pediatric Liver Diseases and Filfoie, DHU Hepatinov, National Center for Rare Liver Diseases, FILFOIE, Paris, France
Search for more papers by this authorDominique Debray
Pediatric Hepatology Unit, APHP-University Hospital Necker-Enfants Malades, Paris V University, Paris, France
Search for more papers by this authorFlorent Guérin
Paediatric Surgery Unit, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Paris, France
Search for more papers by this authorMaurice Beghetti
Pediatric Cardiology Unit, Centre Universitaire de Cardiologie et Chirurgie Cardiaque Pédiatrique, University Hospitals of Geneva and Lausanne, Geneva, Switzerland
Search for more papers by this authorLaurent Savale
AP-HP, Service de Pneumologie, Centre de Référence de l’Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin Bicêtre
INSERM UMR-S 999 Hôpital Marie Lannelongue, Le Plessis Robinson
Université Paris–Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France
Search for more papers by this authorBarbara E. Wildhaber
Swiss Pediatric Liver Center, University Center of Pediatric Surgery of Western Switzerland, University Hospitals Geneva and Geneva University, Geneva, Switzerland
Search for more papers by this authorEmmanuel Gonzales
Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases and Folfoie-DHU Hepatinov, Assistance Publique-Hôpitaux de Paris and INSERM UMR-S 1174, Université Paris-Sud, Paris-Saclay, Le Kremlin-Bicêtre, France
Search for more papers by this authorMembers of the International Registry of Congenital Porto-Systemic Shunts
Search for more papers by this authorCorresponding Author
Valérie A. McLin
Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland
Address correspondence and reprint requests to Valérie A. McLin, MD, Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland (e-mail: [email protected]).Search for more papers by this authorStephanie Franchi Abella
Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, University Paris-Sud, Orsay
Pediatric Liver Transplantation Unit, Reference Centre for Pediatric Liver Diseases and Filfoie, DHU Hepatinov, National Center for Rare Liver Diseases, FILFOIE, Paris, France
Search for more papers by this authorDominique Debray
Pediatric Hepatology Unit, APHP-University Hospital Necker-Enfants Malades, Paris V University, Paris, France
Search for more papers by this authorFlorent Guérin
Paediatric Surgery Unit, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Paris, France
Search for more papers by this authorMaurice Beghetti
Pediatric Cardiology Unit, Centre Universitaire de Cardiologie et Chirurgie Cardiaque Pédiatrique, University Hospitals of Geneva and Lausanne, Geneva, Switzerland
Search for more papers by this authorLaurent Savale
AP-HP, Service de Pneumologie, Centre de Référence de l’Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin Bicêtre
INSERM UMR-S 999 Hôpital Marie Lannelongue, Le Plessis Robinson
Université Paris–Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France
Search for more papers by this authorBarbara E. Wildhaber
Swiss Pediatric Liver Center, University Center of Pediatric Surgery of Western Switzerland, University Hospitals Geneva and Geneva University, Geneva, Switzerland
Search for more papers by this authorEmmanuel Gonzales
Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases and Folfoie-DHU Hepatinov, Assistance Publique-Hôpitaux de Paris and INSERM UMR-S 1174, Université Paris-Sud, Paris-Saclay, Le Kremlin-Bicêtre, France
Search for more papers by this authorMembers of the International Registry of Congenital Porto-Systemic Shunts
Search for more papers by this authorThe authors report no conflicts of interest.
ABSTRACT
Congenital portosystemic shunts are increasingly recognized in several settings and at any age. The following are some of the most common presentations: prenatal ultrasound, neonatal cholestasis, incidental finding on abdominal imaging, or systemic complications such as unexplained cardiopulmonary or neurological disease, or the presence of liver nodules in a noncirrhotic liver. The purpose of the present review is to summarize clinical presentation and current recommendations for management, and highlight areas of future research. Illustrative examples from the veterinary literature complement our current lack of knowledge of this rare malformation often masquerading as a multisystem disease.
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