Abstract
A 13-year-old male with unicteric stage of primary sclerosing cholangitis (PSC) manifesting purpura and hepatosplenomegaly was reported. ERC shown features of the biliary system compatible with those of type II of intrahepatic PSC described by Li Yeng et al. Laparoscopy was helpful for the diagnosis of PSC in this case. A deep longitudinal trench-like depression was observed on the surface of the left lobe. Liver surface showed macronodular cirrhosis with a number of lymph cysts mainly on depressed areas. The right hepatic lobe was swollen with regenerative findings. The second laparoscopy carried out ten months later demonstrated a hemispherical regenerative mass on the inferior surface of the medial portion of the right lobe. These laparoscopic features are compatible with those reported as characteristics in an adult type of PSC. Liver consistence was hard when the biopsy was done. Histological examination of the biopsy specimen revealed periductal fibrosis, being compatible with the findings in PSC. It is noted that this case without jaundice had completed cirrhosis. From the laparoscopic observation in this case, a deep longitudinal trench-like depression and formation of regenerative mass seem to be characteristic of PSC.
