Extrahepatic portal hypertension in children

The details of eighteen children with extrahepatic portal hypertension seen during the last twenty years at the Childrens Hospital of Los Angeles are presented. All had bleeding from esophagogastric varices, but in several this was not the presenting symptom or finding. Three initially presented with splenomegaly and five with ascites. Eleven of the patients manifested ascites at some stage of their disease, more commonly during infancy or early childhood. Three older patients currently have ascites which has persisted for two, six, and seven years, respectively. An etiologic mechanism such as that usually ascribed to the development of occlusion of the portal vein was observed in only three of the eighteen children. It is suggested that neonatal omphalitis or peritonitis is an unlikely cause and that the etiologic mechanism is, in fact, unknown. Fever, usually due to a respiratory infection, preceded varical hemorrhage in a number of patients. The implication of this feature is discussed. The results of operative management were disappointing. The ineffectiveness of shunt procedures in eliminating recurrent varical hemorrhage was probably due in large part to the young age of the patients at the time of operation. Of great interest was the observation that in four children varical hemorrhage ceased spontaneously. In two of them, varices are no longer demonstrable on roentgenogram. In the other two, this examination had not been carried out. Six of the eighteen patients are currently over the age of twenty years; only one still has bleeding. These features are consonant with the rarity with which an adult with extrahepatic portal hypertension provides a history of bleeding episodes extending from childhood.