Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease

BackgroundThe objective of our study was to determine if hepatic ultrasound findings in paediatric patients with cystic fibrosis and suspected liver disease are related to histopathological results derived from liver biopsies.MethodsA retrospective analysis of ultrasound and liver biopsy findings using published criteria was performed in 30 children with cystic fibrosis suspected as having liver disease on clinical, biochemical and ultrasonographic criteria. The results were correlated and assessed for intra- and interobserver agreement.ResultsA significant association was found for the prediction of fibrosis or cirrhosis on the basis of ultrasound (p = 0.03). There was no significant association between normal or indeterminate ultrasound and histology results. A high intra- and interobserver variability was found in sonographic assessment of the hepatic echostructure.ConclusionsThe diagnosis of early liver disease in cystic fibrosis cannot reliably be made on the basis of ultrasound alone. A normal ultrasound does not preclude significant liver fibrosis in cystic fibrosis. An abnormal US that suggests cirrhosis predicts the presence of moderate to severe liver disease.