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. 2024 Jul 31;40(1):212.
doi: 10.1007/s00383-024-05785-y.

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

Qi Toh  1 , Yong Chen  1 , Yang Yang Lee  2 , Vidyadhar Padmakar Mali  2 , Suet Cheng Choo  1 , Li Wei Chiang  3
Affiliations

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

Qi Toh et al. Pediatr Surg Int. .

Abstract

Purpose: 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.

Method: 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.

Results: 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.

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

Keywords: Biliary atresia; Cholestatic jaundice; Diagnostic score.

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