Congenital polycystic disease of the liver is a recognized entity.1-7 In occurs in association with cystic disease of other organs, including the kidneys, pancreas, spleen, ovaries, and lungs. Women are more commonly affected than men, and symptoms usually first appear in the fourth or fifth decades.2,3,8,9 The disorder is thought to result from a congenital abnormality of the bile ducts.10 The symptoms are variable and nonspecific, and the function of the liver is rarely impaired.
Polycystic disease confined to the liver does not alter life expectancy. The kidneys are also frequently involved. This was true of 56% of 499 cases reported in 1937 by Davis2,3; in this combination, the symptoms and prognosis are those of the renal lesion. Although gastrointestinal bleeding has been reported in association with polycystic liver disease, the source of hemorrhage has not been established.11,12 Portal hypertension has not heretofore been described.