Advances in the diagnosis of Biliary Atresia

Biliary atresia (BA) poses a significant challenge in pediatric hepatology, constituting a substantial proportion of neonatal cholestasis cases and serving as a leading indication for liver transplantation in children. Timely identification and accurate diagnosis are paramount in improving outcomes for affected infants.

Efficient screening methods, such as infant stool color charts and measuring conjugated bilirubin levels in newborn blood samples, play a crucial role in detecting neonatal cholestasis and BA early. Other diagnostic tests, including ultrasonography (USG), hepatobiliary iminodiacetic acid (HIDA) scan, cholangiography, liver biopsy, and elastography, aid in confirming BA diagnosis.

Recent studies have underscored the utility of shear wave elastography (SWE) as an adjunct to USG, with consideration of age-based cut-offs. However, the use of SWE may be limited in the setting of equivocal USG findings. 

Liver fibrosis occurs with BA pathogenesis, thus necessitating accurate fibrosis assessment in disease management. While 2D SWE demonstrates promising sensitivity and specificity in detecting advanced fibrosis and cirrhosis in BA, the heterogeneity of non-BA neonatal cholestasis etiologies warrants careful consideration in interpretation. Additionally, SWE shows potential in predicting BA outcomes post-Kasai Portoenterostomy when integrated with clinical parameters and gene expression profiles.

In conclusion, initiating USG assessment complemented by adjunctive tools like SWE helps enhance BA diagnosis and prognostication. Although SWE is a valuable adjunct in fibrosis assessment and outcome prediction, it should be integrated with established diagnostic modalities for optimal clinical management of BA.

Srivastava A. Improving the Outcome of Biliary Atresia: All Hands on Deck. Indian J Pediatr. 2024. https://doi.org/10.1007/s12098-024-05102-4