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Surgical treatment of the congenital portocaval fistula in children

T. Blanc, S. Franchi-Abella, S. Branchereau, V. Lambert, O. Soubrane, D. Pariente, O. Bernard, F. Gauthier

Department of Pediatric Surgery, Paris Sud Faculty of Medicine, Kremlin – Bicetre Hospital, Paris, France



Introduction: The congenital porto-caval fistulas (CPCF) can cause encephalopathy, arterio-venous pulmonary shunts (AVPS), pulmonary arterial hypertension (PAHT) or hepatic masses, requiring conservative vascular treatment or even liver transplantation. The preventive or curative treatment can be achieved by interventional radiology, by surgery or by the association of both. The aim of this study is to report our experience with the conservative surgical treatment.

Material and methods: The files of the children operated on for CPCF by the same surgical team, from 1997 to 2006 were retrospectively reviewed.

Results: Nine children (5 boys) with age range at diagnosis from 2 months to 17 years old (mean age: 7 years) were operated upon. Five children were asymptomatic and 4 had encephalopathy. One had AVPS, 3 had PAHT and 6 had one more multiple liver masses. The anatomic study by ultrasonography, CT angiography, MRI and direct phlebography with attempt of fistula occlusion showed 3 types of CPCF: i) persistent ductus venosus (n=2), ii) transparenchymatous fistula into the retrohepatic IVC (n=3) and iii) fistula into the suprarenal IVC (n=4). The hepatic portal system was hypoplastic in types i) and ii),it was not visible for type iii). Three fistulas were closed in one-stage surgery. For the other six the cross-clamping of the fistula was not tolerated and were treated by cerclage and delayed obliteration (surgically n=4, radiological n=2). Vascular re-expansion of the hepatic portal system was achieved in all cases, through the portal vein in type i) and ii), through a cavernoma without portal hypertension in type iii).

Conclusion: By means of a precise anatomical study and experience of surgery and interventional radiology, the one or two-stage closure of the CPCF can lead to a satisfactory portal reperfusion. The liver transplant is not indicated as first line of treatment of the CPCF.


Key words: congenital porto-caval fistula, arterio-venous pulmonary shunt, pulmonary arterial hypertension, interventional radiology, surgical closure