Portal hypertension in fibrocystic disease of the pancreas.

It is now known that the liver is often damaged in fibrocystic disease of the pancreas. Bodian (1952) found patchy portal fibrosis and an increase in bileduct structures, which he termed focal biliary fibrosis, in half of the 50 cases in his series in which histological sections of liver were available. This lesion is found most often and most severely in older children, but Claireaux (1956) has recorded similar findings in the liver of a newborn infant with fibrocystic disease. The livers in Bodian's series were not deeply scarred and there was little evidence of portal hypertension. Pugsley and Spence (1949) reported a grossly nodular liver in fibrocystic disease with a normal spleen, but in a series of 116 patients with the disease Webster and Williams (1953) noted splenic enlargement in the five cases with clinical evidence of cirrhosis. Di Sant' Agnese (1955) recorded two instances of cirrhosis and portal hypertension among a number of cases with normal or partial pancreatic function, and a recent leading article in the British Medical Journal (1956) also draws attention to this complication, but it is rare. In the case presented here great enlargement of the spleen resulted from portal hypertension and necessitated treatment, although the primary condition was otherwise in a satisfactory state of control.