title: [Congenital portosistemic shunt. Importance of early treatment].
abstract: OBJECTIVE
  Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition.
  
  
  METHODS
  Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007. The mean age at diagnosis was 5.5 years (1 month-15 years). Five patients had associated comorbidities.
  
  
  RESULTS
  Five patients had developed hyperammonemia and intellectual impairment, one of those manifested with an onset of coma. Four patients have developed at diagnosis liver tumors, including focal nodular hyperplasia/regenerative nodules (n=3) and adenomas (n=3). One patient with multiple tumors required a hepatectomy owing to compressive symptoms. Two patients, developed malignant degeneration, a child under five years treated with liver transplantation and another in adulthood treated with hepatectomy. In one patient, diagnosed in the neonatal period, the shunt occlusion occurred spontaneously. Direct portography with the occlusion test was performed in five patients, the shunt was occluded with interventional radiology in three cases, surgery in one and liver transplantation in the remaining.
  
  
  CONCLUSIONS
  The treatment of the SPSC must be early to prevent and even to reverse its complications, avoiding liver transplantation. Currently, interventional radiology is essential in the strategy to follow and treatment of these patients.
authors: Servicio de Cirugía Pediátrica. Hospital Universitario Vall D´Hebron. Barcelona.
authors: N
authors: N
authors: Vicente
authors: Servicio de Radiología Intervencionista. Hospital Universitario Vall D´Hebron. Barcelona.
authors: M
authors: M
authors: Pérez
authors: Servicio de Cirugía Pediátrica. Hospital Universitario Vall D´Hebron. Barcelona.
authors: R
authors: R
authors: Gander
authors: Servicio de Radiología Intervencionista. Hospital Universitario Vall D´Hebron. Barcelona.
authors: A
authors: A
authors: Segarra
authors: Servicio de Cirugía Pediátrica. Hospital Universitario Vall D´Hebron. Barcelona.
authors: C
authors: C
authors: Leganés
authors: Servicio de Cirugía Pediátrica. Hospitales Universitarios Virgen del Rocío y Virgen Macarena. Sevilla.
authors: J
authors: J
authors: Bueno
keywords: Abernethy malformation; Congenital portosystemic shunt