A rare hereditary disease of the liver (fibrocholangiocystosis--congenital fibrosis of the liver) was surgically treated in 43 children. A conclusion is made that different palliative operations are inexpedient. Ilio-cavamesenterial anastomoses may be recommended for preventing relapses of gastroesophageal hemorrhages. These operations should be performed at the age of 5-10 years. After palliative operations recurrent hemorrhages were noted in 68,4%, lethality in 26,3%. After vascular anastomoses recurrent hemorrhages were found in 23,3%, lethality--in 7,7%.

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