The intrahepatic ductal dilatation resembles Caroli's disease. The pancreatic abnormality consists of dilated duct, cysts and fibrosis with diminution of parenchymal tissue. Clinically the child develops during the neonatal period cholestatic jaundice followed by renal and pancreatic failure. Ivemark syndrome should not be confused with the asplenia-cardiac anomaly syndrome also bearing the same eponym. Ultrasound findings in the three affected organs suggest the diagnosis that unfortunately will be confirmed at autopsy.
Management is supportive.
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