engleski

The prognostic value of ductal plate malformation in biliary atresia

Objectives and Study: The intrahepatic bile ducts develop from the fetal ductal plate through a process called ductal plate remodeling. Disturbances in this process give rise to ductal plate malformation (DPM) which has been observed in some cases of biliary atresia (BA). We have evaluated the presence of DPM as an indicator of prognosis, along with ductular proliferation, Ishak score and age at operation. Methods: Between 1984 and 2008, 38 patients with BA underwent hepatoportoenterostomy (HPE). Three patients were lost to follow-up. We analyzed 28 biopsy specimens for presence of DPM using cytokeratin 19 staining. DPM was present when a concentric cellular arrangement was detected. Outcome at 3 months and 2 years after HPE and SNL was calculated. Patients with onset of cholestasis with acholic stools in the first week of life and/or associated congenital anomalies were assigned in the fetal, and the others were in the perinatal group. Results: Eight out of 28 patients (28.6%) had DPM and 20 were DPM-negative. Duration of follow up ranged from 5.7 to 220.3 months, with a mean age of 73.2 months. Twelve (42.9%) patients were male, and 16 (57.1%) were female. Even though DPM-negative patients had lesser grades of fibrosis, and lesser extent of ductular proliferation they have less favourable outcome at 3 months and 2 years after HPE (Fisher exact test) and shorter SNL (log rank test). There was difference in terms of fetal/perinatal ratio between the DPM- positive and negative group, but it didn’t reach statistical significance. None of our patients with fetal type BA had DPM, and all of them had poor outcome. There was no difference between the DPM-positive and negative patients in perinatal group regarding any outcomes. Conclusion: Our results do not support thesis that presence of DPM is correlated with unfavourable outcome in patients with BA. It is fetal type of BA atresia, which is associated with shorter SNL and early failure of HPE (p 0.001, log-rank test). In 2 subgroups of patients with perinatal type of BA regardless of DPM presence there was no difference in outcome. In patients with DPM, surgery was performed earlier, and that may have contributed to better outcome

ductal plate malformation; biliary atresia; outcome

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