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Simple biliary atresia score-a validated diagnostic aid for infantile cholestasis
Qi Toh1, Yong Chen1, Yang Yang Lee2
1Department of Pediatric Surgery, KK Women's and Children's Hospital, No. 100, Bukit Timah Road, Singapore, 229899, Singapore.
Insights
A new scoring system helps identify infants with potential biliary atresia (BA). This practical tool aids in early diagnosis and treatment of infantile cholestasis, improving outcomes for newborns.
Area of Science:
- Pediatric Gastroenterology
- Neonatology
- Diagnostic Imaging
Background:
- Infantile cholestasis diagnosis can be delayed due to variable workups.
- Delayed diagnosis of biliary atresia (BA) in infants leads to poorer outcomes.
- A need exists for a practical tool to stratify risk for BA in jaundiced infants.
Purpose of the Study:
- To develop and validate a practical scoring system for risk stratification of infantile cholestasis.
- To identify infants with a high likelihood of biliary atresia (BA).
- To expedite the diagnosis and management of BA in neonates.
Main Methods:
- A score (0-7) was developed using logistic regression from a retrospective cohort (n=58).
- The score incorporates gallbladder length, common bile duct diameter, portal vein echogenicity, direct-to-total bilirubin ratio (D/T), and gamma-glutamyl transferase (GGT).
- The score was validated in a separate cohort (n=28), with final diagnoses confirmed by intraoperative cholangiogram and liver histopathology.
Main Results:
- A cutoff score of ≥3 demonstrated high sensitivity (100% in derivation, 94% in validation) for diagnosing BA.
- The direct-to-total bilirubin ratio (D/T) was the most sensitive parameter (93%), while common bile duct (CBD) diameter was most specific (88%).
- The score accurately predicted non-BA cases in 65% and 63% of infants in the derivation and validation cohorts, respectively.
Conclusions:
- A validated, simple, and sensitive diagnostic score for cholestatic infants is proposed.
- This score aims to facilitate earlier risk stratification and management of biliary atresia (BA).
- The tool can aid clinicians in making timely decisions for infants with suspected BA.
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.
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