Abstract
Purpose:
Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates and infants. We evaluate the performance of the Simple BA Scoring System (SBASS) in diagnosing BA.
Methods:
With ethical approval, we performed a prospective, cross-sectional study on all infants with cholestatic jaundice referred to three paediatric surgical tertiary centres from June 2021-December 2022. Diagnosis of BA was confirmed by cholangiography and histological findings. We excluded patients who had excretion of hepatic iminodiacetic acid, and those without all SBASS score parameters available. SBASS scoring was applied to all patients and scores were compared to the eventual diagnosis. The score consists of two ultrasonographic findings (gall bladder length < 1.6cm score 1, presence of triangular cord sign score 1), and two laboratory variables (conjugated bilirubin:total bilirubin ratio > 0.7 score 2, gamma-glutamyl transferase(GGT) > 200 U/L score 2). The maximum score was 6.
Results:
Of 97 patients referred, 73 were included in this study: 46 (63%) male, 64(87%) term babies. Fifty-two (71%) had BA (age 57 days [range 45–68]) while 21(29%) were non-BA (age 59 days, [range 54–68]). In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15(72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the scoring system showed a sensitivity of 96.2%, specificity of 61.9% positive predictive value of 86.2%, negative predictive value of 86.7%, and overall accuracy of 86.3%. GGT had the highest sensitivity (94.2%) of the four clinical parameters, while triangular cord sign was the most specific (95.2%) in diagnosing BA.
Conclusion:
Our study shows that the SBASS provides a bedside, non-invasive scoring system to differentiate BA from other cholestatic jaundice causes in infants. Utilizing this scoring system allows for the exclusion of BA using a low risk method and reduces the likelihood of negative surgical explorations.