Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.
OBJECTIVE
Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature.


METHODS
Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods.


RESULTS
Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications.


CONCLUSIONS
Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.
Franchi-Abella
Stéphanie
S
Radiologie Pédiatrique, Hôpital Bicêtre, APHP, Le Kremlin-Bicêtre, France.
Branchereau
Sophie
S
Lambert
Virginie
V
Fabre
Monique
M
Steimberg
Clarisa
C
Losay
Jean
J
Riou
Jean-Yves
JY
Pariente
Danièle
D
Gauthier
Frédéric
F
Jacquemin
Emmanuel
E
Bernard
Olivier
O
D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000209:etiology; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology; D006333:Heart Failure / Q000209:etiology; D006501:Hepatic Encephalopathy / Q000209:etiology; D020065:Hepatopulmonary Syndrome / Q000209:etiology / Q000601:surgery; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000098:blood supply* / Q000601:surgery; D008113:Liver Neoplasms / Q000209:etiology; D008297:Male; D017082:Portal Pressure; D011168:Portal System / Q000002:abnormalities* / Q000601:surgery*; D011169:Portal Vein / Q000002:abnormalities / Q000601:surgery*; D011170:Portasystemic Shunt, Surgical; D016896:Treatment Outcome; D016157:Vascular Fistula / Q000150:complications / Q000151:congenital* / Q000601:surgery; D014682:Vena Cava, Inferior / Q000002:abnormalities*