Simple biliary atresia score-a validated diagnostic aid for infantile cholestasis

The workup of jaundiced infants may be variable and protracted, thereby delaying the diagnosis and timely intervention for biliary atresia (BA). This potentially leads to inferior outcomes. We developed a practical score to stratify infantile cholestasis according to the risk of having BA.

The score (0-7) [gallbladder length ≤ 15 mm (+ 1), common bile duct (CBD) diameter < 0.5 mm(+ 1), pre-portal vein (PV) echogenicity(+ 1), direct-to-total bilirubin ratio (D/T) ≥ 0.7(+ 2), and gamma-glutamyl transferase (GGT) ≥ 200 IU/L(+ 2)] are derived from logistic regression of data from a retrospective cohort of cholestatic infants (n = 58, 41 BA) in our institution. It was then validated with a separate retrospective cohort (n = 28, 17 BA) from another institution. Final diagnoses were as per intraoperative cholangiogram (IOC) and liver histopathology.

A cutoff score of ≥ 3 diagnosed BA with 100% and 94% sensitivity in the derivative cohort (area under receiver operating characteristic curve, AUROC 0.869) and validation cohort (AUROC 0.807), respectively. D/T ratio was the most sensitive (93%) and CBD diameter was the most specific (88%) parameter. The score accurately predicted non-BA in 11(65%) and 7(63%) infants in the derivative and validation cohorts, respectively, with one missed BA in the latter.

We propose a validated, simple, yet sensitive diagnostic score to risk-stratify cholestatic infants, aiming to expedite definitive management of BA.