Treatment

Bleeding episodes are often self-limited.

A cavomesenteric shunt may be useful in younger children, since the vessels are small.

Older individuals may be given sclerotherapy. If bleeding remains uncontrolled, then a distal splenorenal shunt should be performed.

Splenectomy alone has no permanent effect and sacrifices the splenic vein which may be needed at a later date. The procedure is never indicated in portal vein occlusion because there is a 90% rebleeding rate. Also, postsplenectomy sepsis is not uncommon.

Repeated severe haemorrhages should be treated by transendoscopic sclerosis. Oesophagogastrectomy with colon interposition is a last resort.