The long term outcome of 21 children with extrahepatic portal hypertension secondary to portal vein thrombosis managed by surgical intervention was evaluated. Portosystemic shunts, used primarily in nine patients (eight central splenorenal, one mesocaval) after conservative treatment had failed, had no associated mortality and a 56% patency rate. Five of these shunted patients had no further bleeding episodes and did not show encephalopathic impairment. Direct attack procedures-portoazygos operation (four patients) was associated with significant complications, including one fatality. Other direct approaches-oesophageal transection and variceal plication (five patients) had variable outcome. Splenectomy alone (three patients) ameliorated hypersplenism; however, further surgery for recurrent haemorrhage (two patients) was necessary. Endoscopic sclerotherapy controlled recurrent variceal bleeding (three patients) when it became available to the unit. Conservative treatment practised in five children had little success: two patients survived, two died from further haemorrhage, and one was lost to follow up. These results suggest that in centres without endoscopic expertise, and for patients who are sclerotherapy 'failures', surgery can be performed safely and achieve reasonable long term success rates in childhood extrahepatic portal hypertension.
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