We prospectively studied 21 consecutive patient with extrahepatic port
al venous obstruction for evidence of biliary tract disease. Two patie
nts were firs seen with extrahepatic cholestasis; another had recurren
t cholangitis. All three patients with clinical manifest biliary disea
se were adults. Another five patients had icterus on clinical examinat
ion. Live function tests revealed elevated bilirubin levels in 1 patie
nts (66.6%), elevated alkaline phosphatase level in 17 (80.9%) and ele
vated serum ALT levels in (38.0%). Endoscopic retrograde cholangiograp
hy revealed abnormal findings in 17 patients (80.9%). The changes invo
lved the common bile duct (66.6%) more often than they did the hepatic
bile ducts (38.1%). Cholangiographic abnormalities included stricture
s (52.4%), caliber irregularity (23.8%), segmental upstream dilatation
(42.8%), ectasia (9.5%), collateral veins causing extraluminal bile d
uct impressions (14.3%), displacement of ducts (9.5%), angulation of d
ucts (4.7%) and pruning of intrahepatic ducts (9.5%). The pathogenesis
of such cholangiographic abnormalities is unknown. However, possible
factors in such changes include collateral veins bridging the blocked
portal vein, causing bile duct impressions; fibrous scarring of porta
hepatis, causing angulation of bile duct; and ischemic injury to bile
duct, leading to stricture formation and caliber irregularity. Biliary
disease is important in the clinical outcome of patients with extrahe
patic portal venous obstruction because variceal sclerotherapy has pro
longed the life expectancies of such patients.