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Published on: August 23, 2022
Ductal plate malformation in patients with biliary atresia
Jurica Vuković1, Ruža Grizelj, Katarina Bojanić
1Department of Pediatrics, University of Zagreb Medical School, University Hospital Centre Zagreb, Zagreb, Croatia.
Insights
Early jaundice in infants with biliary atresia (BA) indicates a poor prognosis, irrespective of ductal plate malformation (DPM) presence. This finding is crucial for assessing outcomes after hepatoportoenterostomy (HPE) surgery.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Surgical Pathology
Background:
- Ductal plate malformation (DPM+) in biliary atresia (BA) signifies early intrauterine disease and poor prognosis.
- Hepatoportoenterostomy (HPE) is a surgical procedure for BA, and understanding prognostic markers is crucial.
Purpose of the Study:
- To evaluate the prognostic value of DPM in infants with BA following HPE.
- To correlate DPM presence and jaundice onset timing with post-HPE outcomes.
Main Methods:
- Retrospective review of 28 BA infants undergoing HPE.
- Histopathological analysis for DPM presence (DPM+ vs. DPM-).
- Categorization of jaundice onset as early (fetal) or late (perinatal).
Main Results:
- No fetal BA patients achieved jaundice clearance at 3 months post-HPE.
- Jaundice clearance occurred in 5/8 DPM+ and 4/12 DPM- perinatal BA patients (p=0.03).
- Median survival with native liver (SNL) was significantly longer for DPM+ perinatal BA (148.2 months) vs. DPM- perinatal BA (93.2 months) and fetal BA (8.6 months) (p<0.001).
Conclusions:
- Early jaundice onset, irrespective of DPM status, is the strongest predictor of poor outcomes in BA infants post-HPE.
- While DPM presence did not significantly alter outcomes in perinatal BA, early jaundice remains a critical prognostic indicator.
Unlabelled:
The presence of ductal plate malformation (DPM+) on liver histology in children with biliary atresia (BA) is a marker of early intrauterine disease onset and an indication of an unfavorable prognosis. We studied the prognostic value of DPM in infants with BA after hepatoportoenterostomy (HPE). We reviewed 28 BA patients who underwent HPE in a single medical center. We examined the time of jaundice onset after delivery (conjugated hyperbilirubinemia): early onset (fetal phenotype with no jaundice-free interval) vs. late onset (perinatal phenotype with jaundice-free interval) and the presence or absence of DPM (DPM+ or DPM-) histopathology. Primary outcome was jaundice clearance at 3 months after HPE and survival with native liver (SNL). Eight children had fetal and 20 had perinatal BA (8 DPM+, 12 DPM-). At 3 months after HPE, no patients with fetal BA had achieved jaundice clearance, while jaundice clearance was achieved in five patients with DPM+ perinatal disease and four patients with DPM- perinatal BA (P = 0.03, comparing all three groups; P = 0.36, comparing DPM+ vs. DPM- perinatal patients). Median SNL was 8.6 months for fetal BA patients, 148.2 months for DPM+ perinatal BA patients, and 93.2 months for DPM- perinatal BA patients (log-rank test, P < 0.001, comparing all three groups; P = 0.59, comparing DPM+ vs. DPM- perinatal patients). After adjusting for BA type, age older than 2 months at HPE was associated with worse SNL [P = 0.03; hazard ratio = 4.0 (95 % CI, 1.1-14.2)].
Conclusions:
Early onset of jaundice, regardless of DPM histology, was the most ominous sign of poor outcome in infants with BA after HPE.
