PubMed · 973144
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S F Ochsner. 1976. Extrinsic abnormalities affecting the biliary system.. https://doi.org/10.1016/0037-198x(76)90036-5
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Intrahepatic duct (IHD) stone is a common disease in Taiwan. In some cases, left IHD stones cannot be demonstrated by endoscopic retrograde cholangiography (ERC) or even by percutaneous cholangiography (PTC). This study was designed to evaluate the efficacy of sonography in demonstrating left IHD stones in poorly opacified left IHD by either ERC or PTC. Of 109 patients with hyperechoic nodular lesions in either the dilated left IHD or atrophic left lobe of the liver, with or without acoustic shadows, 49 patients were excluded because they refused further study. Among the remainder (60 patients), 33 patients had good opacification of left IHD in ERC or PTC. Stones were seen in 31 patients, and tumor obstruction in 2 patients. Twenty-seven patients had complete or partial absence of the left IHD in cholangiograms. Stones were found at surgery in 23 patients and by CT scan in 3 patients. Mucin-producing cholangiocarcinoma was found in 1 patient. The positive predictive value of the demonstration of stones in left IHD by ultrasound was 95% (57/60). Only 15% of patients with atrophy of the left hepatic lobe and stones were demonstrated by sonogram in this series. We conclude that the detection of left IHD stones by ultrasound in patients with poor opacification of the left IHD in cholangiograms is reliable procedure.
The histological and immunohistochemical characteristics of the liver in 44 children (28 boys, 16 girls) with extrahepatic biliary atresia at different stages of the clinical course were studied. Thirty-four wedge liver biopsy specimens taken during Kasai operations (25 specimens) and relaparotomy (9 specimens) and 20 hepatectomy explants taken at the time of transplantation were examined. Routine histological stains and monoclonal antibodies against different molecular weight cytokeratins and HLA-DR were used. The histopathological changes and the pattern of cytokeratin expression observed during the course of the disease were suggestive of persistent or recurrent extrahepatic biliary obstruction that occurred despite the Kasai operation and eventually led to cirrhosis and liver failure. Quantitative studies showed a progressive loss of intrahepatic bile ducts over the time course of the disease. This destruction of bile ducts had a geographic anatomical distribution in hepatectomy specimens, and in two livers it occurred predominantly in only one lobe. This geographic distribution of the vanishing bile ducts probably indicates an unpredictable and uneven obliteration of bile ducts in the porta hepatis during portoenterostomy wound healing and scarring.
According to our recent study, 38 of 93 patients (40.9%) who underwent portoenterostomy at Juntendo University Hospital between 1977 and 1986 survived for more than 5 years. In order to learn the future improvement of the prognosis of biliary atresia patients who undergo portoenterostomy, we investigated the relationship between the prognosis of biliary atresia patients and the age and the histological changes of liver at the time of surgery. Twenty-seven of 30 patients (90%) who survived for more than 5 years with no jaundice and no findings of liver cirrhosis had a mild degree of liver fibrosis (F1 or F2) and a mild degree of degeneration of intrahepatic bile ducts (B1 or B2) at the time of surgery, in spite of the difference of size of intrahepatic bile ducts at the porta hepatis. On the other hand, all 8 patients who showed severe liver fibrosis (F3) and severe degeneration of intrahepatic bile ducts (B3) did not survive for more than 5 years. Accordingly, we can expect promising prognoses in patients who undergo refined portoenterostomy procedures and receive proper postoperative treatment before they have severe histological changes of liver. The portoenterostomy will still play an important role as treatment of biliary atresia in the future.