2017 Volume 53 Issue 7 Pages 1257-1263
Purpose: We reviewed our experience in treating patients with biliary atresia (BA) associated with vitamin K deficiency bleeding (VKDB) in a single institution to assess their outcomes.
Methods: A retrospective study of 76 patients who underwent a Kasai operation from 1981 to 2016 was carried out. The patients were divided into two groups: group A (n = 9) consisted of patients who showed BA with VKDB, and group B (n = 67) consisted of patients who showed BA without VKDB. The characteristics, clinical parameters, disappearance rates of jaundice and cumulative native liver survival rate were retrospectively assessed. Furthermore, the characteristics of the patients in group A were assessed in detail.
Results: There was no statistically significant difference in the characteristics and clinical parameters between the two groups. The age at the time of Kasai operation was significantly higher and the disappearance rate of jaundice was significantly lower in group A than in group B. Furthermore, the overall 10-year cumulative native liver survival rate was significantly lower in group A than in group B (p < 0.05). Eight of the nine patients in group A were fed with breast milk. The ages at onset of VKDB ranged from 41 to 84 days. Four of these nine group A patients had intracranial hemorrhage. They had no neurological sequelae.
Conclusion: In the patients with BA associated with VKDB, the age at the time of Kasai operation was delayed. These patients tended to have intracranial hemorrhage as a complication and had a poor prognosis. The early detection of BA is essential for preventing VKDB and intracranial hemorrhage.
