Background: Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis.
Methods: A retrospective case note review was performed of all BA patients at a single centre from 1999 to 2016. Demographics and clinical outcome data were collected. Additional data on laparotomy, parenteral nutrition, and referral were collected from patients who underwent PAS. Data are median (range).
Main results: Two-hundred-and-fifty-seven children were reviewed. Of these, 16 (6.2%) underwent PAS on day 3 (0-23), during which 5 atretic gallbladders were noted. Gallbladder appearance was not referenced in the operation notes of 8 infants. Jaundice and acholic stools were noted at 4 (0-56) days and 21 (0-60) days, respectively. Age at KPE was comparable between PAS and the other patients (50 vs. 51days; P=0.78), but native liver survival was significantly lower after PAS (p<0.0001). Mortality rate was higher in PAS patients (25% vs. 4.5%; P=0.0007). Survival was unaffected by early referral of patients on finding an atretic gallbladder at surgery.
Conclusion: About 6% of infants have already undergone abdominal surgery for biliary atresia associated intestinal anomalies. Routine gallbladder examination at time of laparotomy could have aided earlier diagnosis and treatment of biliary atresia in up to 80% of patients in this cohort. However, our data suggest that clinical outcome is poorer in biliary patients who undergo prior abdominal surgery and is not improved by earlier referral.
Level of evidence: Prognostic study: Level III.
Keywords: Biliary atresia; Kasai portoenterostomy; Prognosis, gall bladder.
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