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European Journal of Pediatrics

, Volume 171, Issue 12, pp 1799–1804 | Cite as

Ductal plate malformation in patients with biliary atresia

  • Jurica Vuković
  • Ruža Grizelj
  • Katarina Bojanić
  • Marijana Ćorić
  • Tomislav Luetić
  • Stipe Batinica
  • Mirjana Kujundžić-Tiljak
  • Darrell R. Schroeder
  • Juraj SprungEmail author
Original Article

Abstract

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.

Keywords

Conjugated (direct) hyperbilirubinemia Fetal biliary atresia Hepatoportoenterostomy Jaundice Liver histopathology Perinatal biliary atresia Survival with native liver 

Abbreviations

BA

Biliary atresia

BASM

Biliary atresia splenic malformation

CI

Confidence interval

DPM

Ductal plate malformation

HPE

Hepatoportoenterostomy

SNL

Survival with native liver

Notes

Conflict of interest

The authors do not have a financial relationship with the organization that sponsored the research.

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Jurica Vuković
    • 1
  • Ruža Grizelj
    • 1
  • Katarina Bojanić
    • 1
  • Marijana Ćorić
    • 2
  • Tomislav Luetić
    • 3
  • Stipe Batinica
    • 3
  • Mirjana Kujundžić-Tiljak
    • 4
  • Darrell R. Schroeder
    • 5
  • Juraj Sprung
    • 6
    Email author
  1. 1.Department of PediatricsUniversity of Zagreb Medical School, University Hospital Centre ZagrebZagrebCroatia
  2. 2.Department of Pathology and CytologyUniversity of Zagreb Medical School, University Hospital Centre ZagrebZagrebCroatia
  3. 3.Department of Pediatric SurgeryUniversity of Zagreb Medical School, University Hospital Centre ZagrebZagrebCroatia
  4. 4.Department of Medical Statistics, Epidemiology, and Medical InformaticsAndrija Štampar School of Public Health, University of Zagreb Medical SchoolZagrebCroatia
  5. 5.Division of Biomedical Statistics and InformaticsMayo ClinicRochesterUSA
  6. 6.Department of AnesthesiologyMayo ClinicRochesterUSA

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