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Kasai Portoenterostomy Delays: Is a HIDA Scan Worth the Wait?
Yi Zou Lim1, Usamah Chaudhary2, Yacine Issioui2
1Children's Research Institute at UT Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA.
Insights
Diagnosing biliary atresia (BA) in infants is crucial. While hepatobiliary scintigraphy can avoid surgery for some, it delays Kasai Portoenterostomy (KPE), impacting outcomes.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatal Care
Background:
- Biliary Atresia (BA) is a leading cause of pediatric end-stage liver disease and liver transplantation.
- Accurate and timely diagnosis of BA in neonates is challenging but critical for improved outcomes.
Purpose of the Study:
- To evaluate the diagnostic accuracy of hepatobiliary scintigraphy in suspected BA cases.
- To assess the impact of diagnostic methods on the timing of surgical intervention and patient prognosis.
Main Methods:
- Retrospective cohort study of infants with hyperbilirubinemia and suspected BA.
- Analysis of clinical data including age at presentation, diagnostic procedures (hepatobiliary scintigraphy, operative exploration), and surgical outcomes (Kasai Portoenterostomy - KPE).
- Investigation of liver fibrosis severity and native-liver survival in relation to age at intervention.
Main Results:
- Hepatobiliary scintigraphy identified BA in 17% of cases, while operative exploration diagnosed it in 72%.
- The time from jaundice presentation to KPE was 2.1x longer when hepatobiliary scintigraphy was used.
- Infants undergoing KPE after 30 days of age showed significantly worsened liver fibrosis and poorer outcomes, including higher rates of liver transplantation.
Conclusions:
- Hepatobiliary scintigraphy can help avoid unnecessary surgery in some infants with suspected BA but introduces significant delays to KPE.
- Early intervention (ideally before 30 days of age) is crucial for mitigating liver injury progression and improving prognosis in BA patients.
- The study highlights the trade-off between diagnostic certainty and timely surgical treatment in managing neonatal cholestatic liver disease.
Background:
Biliary Atresia (BA), an obstructive cholangiopathy, is the most common cause of end-stage liver disease and liver transplantation in children. Timely differentiation of BA from other causes of neonatal jaundice remains a challenge, yet is critical to improving outcomes.
Methods:
Clinical characteristics including demographics, age at jaundice presentation, age at hepatobiliary scintigraphy, age at surgery, severity of liver fibrosis, and native-liver survival were reviewed in infants with hyperbilirubinemia and suspected BA for this single center retrospective cohort study. We investigated the accuracy of hepatobiliary scintigraphy as well as elapsed time from jaundice presentation to diagnostic intervention.
Results:
BA was suspected in 234 infants. BA was identified in 17% of infants with hepatobiliary scintigraphy and 72% of infants who underwent operative exploration without hepatobiliary scintigraphy. Elapsed time from jaundice presentation to Kasai Portoenterostomy (KPE) for BA patients was 2.1x longer if hepatobiliary scintigraphy was obtained (p = 0.084). The mean age at KPE for this cohort was 66.8 days (n = 54), with a significantly higher mean age at KPE (75.2 days) for infants who were later listed or underwent liver transplantation (p = 0.038). Histologically, the lowest liver fibrosis scores were seen in infants undergoing KPE <30 days old and worsened significantly with increased age (p < 0.001).
Conclusion:
Hepatobiliary scintigraphy compared to operative exploration for the diagnostic evaluation of infants with suspected BA introduces significant time delays to KPE but enables avoidance of surgery in some infants. The temporal pattern of worsening cholestatic liver injury from BA with each day of increased age highlights the importance of intervening as early as possible for the best prognosis.
Type Of Study:
Retrospective study, Level of evidence: III.
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