title: [Congenital portosystemic shunts: experience of the western region of France over 5 years].
abstract: BACKGROUND
  Congenital portosystemic shunts (CPS) are rare vascular malformations that lead to liver metabolic modifications. The aim of this study was to describe the management and outcome of CPS in children who were followed in the western region of France and to compare our experience to a literature review.
  
  
  METHODS
  This retrospective study reports nine children with CPS who were followed in the western region of France between 2008 and 2013.
  
  
  RESULTS
  Among these nine children, six were detected by prenatal ultrasonography, one had cholestatic jaundice during the postoperative period, one had cholestatic jaundice during the first days of life, and one had dyspnea and fatigue. Two children had liver tumors. The CPS was intrahepatic for four children and extrahepatic for two children. Three others had a patent ductus venosus. Among the intrahepatic shunts that were discovered before the age of 2 years, three closed spontaneously. The five other shunts were closed surgically or by interventional radiology techniques, after angiography for four children. After closure of eight shunts, the outcome was favorable for six children. Two children presented complications after surgery: portal hypertension and portal thrombus.
  
  
  CONCLUSIONS
  When CPS is suspected, the diagnosis should be confirmed and complications should be investigated. Shunt closure restores portal flow and prevents complications.
authors.affiliation: Service de pédiatrie, hôpital mère-enfant, CHU de Nantes, 7, quai Moncousu, 44093 Nantes cedex 1, France. Electronic address: gaelle.hubert44@gmail.com.
authors.forename: G
authors.initials: G
authors.lastname: Hubert
authors.affiliation: Service de pédiatrie, CHU d'Angers, 4, rue Larrey, 49033 Angers cedex 9, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Giniès
authors.affiliation: Service de pédiatrie, CHU de Rennes, 16, boulevard de Bulgarie, 35056 Rennes cedex 9, France.
authors.forename: A
authors.initials: A
authors.lastname: Dabadie
authors.affiliation: Cabinet de pédiatrie, 15, quai Lamennais, 35000 Rennes, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Tourtelier
authors.affiliation: Service de pédiatrie, CHU de Tours, 49, boulevard Béranger, 37049 Tours cedex 9, France.
authors.forename: S
authors.initials: S
authors.lastname: Willot
authors.affiliation: Service de radiologie pédiatrique, pôle imagerie et médecine nucléaire, hôpital Bicêtre, 78, rue du Général-Leclerc, 94270 Kremlin-Bicêtre, France.
authors.forename: D
authors.initials: D
authors.lastname: Pariente
authors.affiliation: Service de radiologie pédiatrique, CHU de Nantes, 7, quai Moncousu, 44093 Nantes cedex 1, France.
authors.forename: T
authors.initials: T
authors.lastname: Lefrançois
authors.affiliation: Service de pédiatrie, hôpital mère-enfant, CHU de Nantes, 7, quai Moncousu, 44093 Nantes cedex 1, France.
authors.forename: D
authors.initials: D
authors.lastname: Caldari
mesh_terms: D000293:Adolescent; D000792:Angiography; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007565:Jaundice / Q000209:etiology; D007567:Jaundice, Neonatal / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D011247:Pregnancy; D012075:Remission, Spontaneous; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D016216:Ultrasonography, Prenatal; D054079:Vascular Malformations / Q000175:diagnosis* / Q000628:therapy*