title: Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient.
abstract: BACKGROUND
  Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.
  
  
  METHODS
  A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given.
  
  
  RESULTS
  According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt.
  
  
  CONCLUSIONS
  Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Gloria
authors.initials: G
authors.lastname: Chocarro
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: María Virginia
authors.initials: MV
authors.lastname: Amesty
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Encinas
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Alejandra
authors.initials: A
authors.lastname: Vilanova Sánchez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Hernandez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Ane M
authors.initials: AM
authors.lastname: Andres
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Manolo
authors.initials: M
authors.lastname: Gamez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Juan Antonio
authors.initials: JA
authors.lastname: Tovar
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Lopez Santamaria
mesh_terms: D058070:Asymptomatic Diseases; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*