EXTRAHEPATIC PORTAL VENOUS OBSTRUCTION IN CHILDHOOD - ETIOLOGY, CLINICAL AND LABORATORY FINDINGS AND PROGNOSIS OF 34 PATIENTS

Extrahepatic obstruction of the portal vein is a well known cause of p ortal hypertension in childhood, that causes severe morbidity. We eval uated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5 +/- 3.8 years) with this diagnosis, to define the clinical pictur e, laboratory changes, diagnostic tools and therapeutic modalities. Ga strointestinal bleeding was the commonest mode of presentation (64.7%) , with the second being splenomegaly. The cause of the obstruction cou ld be determined in 38.2% (13/34) of the subjects. At the beginning of the study the main diagnostic procedure was splenoportography althoug h in more recent years pulsed duplex Doppler ultrasonography has been used. The follow up period was a median of 5 years (range i-11 years). The mean number of bleeding episodes was 4.7 +/- 5.9 (range 1-26), wh ile nine patients never bled. There was no mortality. Ten patients und erwent surgery, while sclerotherapy was performed on 10. Twenty-one pa tients received beta-blocker drugs. No difference was found among thes e therapeutic modalities. It is well established that the major risk f or children with extrahepatic portal vein obstruction is gastrointesti nal bleeding which is tolerated quite well. Surgery should be indicate d only in children where bleeding cannot be controlled by medical mean s including sclerotherapy.