TY - JOUR
T1 - Kasai Portoenterostomy Delays
T2 - Is a HIDA Scan Worth the Wait?
AU - Lim, Yi Zou
AU - Chaudhary, Usamah
AU - Issioui, Yacine
AU - Corbitt, Natasha
N1 - Publisher Copyright:
© 2023 Elsevier Inc.
PY - 2023/8
Y1 - 2023/8
N2 - 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.
AB - 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.
KW - Biliary atresia
KW - HIDA
KW - Hepatobiliary scintigraphy
KW - Intra-operative cholangiogram
KW - Liver fibrosis
UR - http://www.scopus.com/inward/record.url?scp=85148722842&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85148722842&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2023.01.003
DO - 10.1016/j.jpedsurg.2023.01.003
M3 - Article
C2 - 36754771
AN - SCOPUS:85148722842
SN - 0022-3468
VL - 58
SP - 1476
EP - 1482
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 8
ER -