The long term outcome of 21 children with extrahepatic portal hyperten
sion secondary to portal vein thrombosis managed by surgical intervent
ion was evaluated. Portosystemic shunts, used primarily in nine patien
ts (eight central splenorenal, one mesocaval) after conservative treat
ment had failed, had no associated mortality and a 56% patency rate. F
ive of these shunted patients had no further bleeding episodes and did
not show encephalopathic impairment. Direct attack procedures - porto
azygos operation (four patients) was associated with significant compl
ications, including one fatality. Other direct approaches oesophageal
transection and variceal plication (five patients) had variable outcom
e. Splenectomy alone (three patients) ameliorated hypersplenism; howev
er, further surgery for recurrent haemorrhage (two patients) was neces
sary. Endoscopic sclerotherapy controlled recurrent variceal bleeding
(three patients) when it became available to the unit. Conservative tr
eatment practised in five children had little success: two patients su
rvived, 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 pe
rformed safely and achieve a reasonable long term success rates in chi
ldhood extrahepatic portal hypertension.