[Hepatic fibropolycystic disease in Mexico. Study of 82 cases]

Abstract

We analyzed 82 cases of hepatobiliary fibropolycystic disease (FPD) that were seen at the Instituto Nacional de la Nutrición "Salvador Zubirán" in Mexico City in the thirty-year period comprised from 1956 to 1986. The different entities that compose FPD were distributed as follows: 61 (74%) cases of polycystic liver disease, 13 (16%) cases of choledochal cyst, and 8 (10%) cases of congenital hepatic fibrosis; there were 5 (6%) cases of Caroli's disease, 3 associated with congenital hepatic fibrosis and 2 with choledochal cyst. Polycystic liver disease predominated in females (67%) and presented at 54 +/- 12 years (mean +/- SEM) with pain, a mass, symptoms related to renal insufficiency or incidentally; polycystic kidneys were present in 61%. Liver function tests were normal in 94%. Choledochal cyst also predominated in females and presented at a mean age of 19 years with cholangitis. Liver function tests were abnormal in 69%. Congenital hepatic fibrosis (50% male) presented with variceal hemorrhage or cholangitis (in 3 patients associated with Caroli's disease). Polycystic kidneys were present in five patients. Four of the five patients with Caroli's disease were female and presented at a mean age of 19 years with cholangitis. It never presented as an isolated disease, but was associated more frequently to congenital hepatic fibrosis. The diseases that are part of the hepatobiliary polycystic disease vary in severity and thus the prognosis in an individual patient is determined by the type of fibropolycystic disease present. This is the largest series of this disease published in our country.