Pulmonary hypertension associated with portal hypertension in childhood. Case report of a 6-year-old child and review of the literature.

Pulmonary hypertension is a rare complication of portal hypertension. Reports of childhood cases especially rare. This report describes an autopsy case of a 6-year-old boy with congenital biliary atresia followed by liver cirrhosis in whom severe hypertensive pulmonary arterial changes, including medial hypertrophy, intimal fibrosis and plexiform lesions were demonstrated. Fresh and organizing fibrin-platelet thrombi as well as probable organized thrombi with recanalization were occasionally found in the pulmonary vasculature, but it was thought that they had probably been formed locally as a late complication rather than being of thromboembolic origin. Retrospectively, the chest roentgenograms had revealed abnormalities suggestive of pulmonary hypertension since infancy, but the patient showed no apparent symptoms of it during life. Previously reported childhood cases of pulmonary hypertension associated with portal hypertension were briefly reviewed. Although the mechanism is presently not known, it is suggested that patients with portal hypertension, even in early childhood, are at risk of developing this unusual complication.