Fifty one children with a diagnosis of fibrocholangiocystosis (FCC) were examined. A degree of circulatory blockade in the portal vein system in FCC was established to be one of the factors conditioning a severity of the course of the disease and its prognosis. With increase in duration of the disease and age of a child, the cirrhotic process in the liver and its complications are aggravating. The esophagogastric bleeding with the incidence as well depending on duration of the disease is the most severe and life-threatening complication. Our experience with operative treatment of FCC is indicative of the necessity to perform early interventions, and of a high effectiveness of creating the vascular anastomosis with preservation of the spleen.
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