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Congenital Portosystemic Shunts

Current Diagnosis and Management

McLin, Valérie A.*; Franchi Abella, Stephanie†,‡; Debray, Dominique§; Guérin, Florent||; Beghetti, Maurice; Savale, Laurent#,**,††; Wildhaber, Barbara E.‡‡; Gonzales, Emmanuel§§ on Behalf of the Members of the International Registry of Congenital Porto-Systemic Shunts

Journal of Pediatric Gastroenterology and Nutrition: May 2019 - Volume 68 - Issue 5 - p 615–622
doi: 10.1097/MPG.0000000000002263
Invited Review
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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.

*Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland

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

§Pediatric Hepatology Unit, APHP-University Hospital Necker-Enfants Malades, Paris V University

||Paediatric Surgery Unit, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Paris, France

Pediatric Cardiology Unit and Centre Universitaire de Cardiologie et Chirurgie Cardiaque Pédiatrique, University Hospitals of Geneva and Lausanne, Geneva, Switzerland

#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

‡‡Swiss Pediatric Liver Center, University Center of Pediatric Surgery of Western Switzerland, University Hospitals Geneva and Geneva University, Geneva, Switzerland

§§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.

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: valerie.mclin@hcuge.ch).

Received 12 July, 2018

Accepted 12 December, 2018

The authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,