MASSIVE FETAL PERICARDIOMEGALY CAUSING PULMONARY HYPOPLASIA, ASSOCIATED WITH INTRA-PERICARDIAL HERNIATION OF THE LIVER

The clinical features, investigation, treatment and outcome of four ne wly born babies with the following recognisable triad of findings are presented: Bilateral pulmonary compression with or without hypoplasia. Massive pericardial effusion without cardiac compromise. An intraperi cardial hernia containing part of the liver. The primary event in the causation of this triad is a congenital defect in the central tendon o f the diaphragm. Compromised hepatic venous outflow involving the hern iated part of the liver is the postulated origin of the fluid within t he pericardium (Budd-Chiari-like effect). Although rare, this triad is clinically identifiable. Sonar imaging clinches the diagnosis. Surgic al correction is simple but the prognosis depends on the presence of p ulmonary hypoplasia which caused death in two cases and on other descr ibed lethal associated anomalies which were not encountered in the rep orted patients.