Extrahepatic portal hypertension in children. Long-term evaluation.

A long-term evaluation of 43 children with extrahepatic portal hypertension indicates a high success rate and excellent subsequent health when a splenorenal or mesocaval shunt can be performed. On the other hand, makeshift shunts inevitably fail. Direct operations are considerably less successful than standard shunts. Esophagogastric resections with interpositions result in long-term freedom from bleeding in only about half the cases, and portoazygous disconnection procedures have been uniformly disappointing. Sclerotherapy in a relatively recent experience has been quite successful, but long-term results are presently unavailable. The condition carries a significant mortality rate. Complications from failed operations, division of the vena cava, and multiple transfusions are numerous. The general health of long-term survivors is excellent in those with successful operations, and is surprisingly good for patients whose operations have been unsuccessful and for those who have had no operations.