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. 2018 Jul 10:73:e289.
doi: 10.6061/clinics/2018/e289.

Current management of biliary atresia based on 35 years of experience at a single center

Wagner de Castro Andrade  1 , Marcos Marques Silva  1 , Ana Cristina Aoun Tannuri  1 , Maria Merces Santos  1 , Nelson Elias Mendes Gibelli  1 , Uenis Tannuri  1
Affiliations

Current management of biliary atresia based on 35 years of experience at a single center

Wagner de Castro Andrade et al. Clinics (Sao Paulo). .

Abstract

Objective: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy.

Methods: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016.

Results: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%.

Conclusion: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

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Conflict of interest statement

No potential conflict of interest was reported.

Figures

Figure 1
Figure 1
Perinatal cholestasis flowchart (TPN – total parenteral nutrition; KP – Kasai portoenterostomy).
Figure 2
Figure 2
Final aspect of the surgery. Note the Roux-en-Y jejunal loop draining the transected porta hepatis.
Figure 3
Figure 3
Kaplan-Meier native liver survival curve (1981-2009 / n=230).
Figure 4
Figure 4
Kaplan-Meier native liver survival curve (2010-2016 / n=27).
See this image and copyright information in PMC

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