Pediatric Biliary Atresia Follow-up

Updated: May 03, 2019
  • Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief Editor: Carmen Cuffari, MD  more...
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Follow-up

Further Outpatient Care

A meta-analysis reported that moderate high-dose steroid therapy improves jaundice clearance, especially for infants who undergo hepatoportoenterostomy by 70 days of age. At the present time, however, and pending more randomized controlled trials with longer follow-up, routine post-Kasai corticosteroid therapy is not recommended.

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Complications

Complications following portoenterostomy in patients with biliary atresia include both acute and chronic problems.

  • In the early postoperative phase, an unsuccessful anastomosis with failure to achieve adequate bile drainage is the most common complication. In this case, adequacy of bile flow may be predicted by the preoperative liver histology and the caliber of bile ductular remnants in the porta hepatis. In one third of all patients, bile flow is inadequate following surgery, and these children succumb to complications of biliary cirrhosis in the first few years of life unless orthotopic liver transplantation is performed.

  • Later in the course, complications related to progressive liver disease and portal hypertension occur in more than 60% of infants who achieved initial surgical success.

  • Cholangitis develops in 50% of patients following portoenterostomy.

  • Hepatocellular carcinoma may be a risk for those patients with cirrhosis and no clinical evidence of portal hypertension. Progressive fibrosis and biliary cirrhosis develop in children who do not drain bile, and liver transplantation is the only option for long-term survival.

  • Detailed management of these complications is described in Histologic Findings, Medical Care, Consultations, Diet, and Medications.

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Prognosis

See the list below:

  • Data regarding outcome from centers worldwide widely vary. The initial success rate of Kasai portoenterostomy (for achieving bile flow) is 60-80%. Clearly, the most critical determinant of outcome remains age at the time of operation. Although individual centers have reported favorable surgical results in some infants older than 3 months, patients are significantly less likely to require early liver transplantation if the portoenterostomy is performed when they are younger than 10 weeks. In the postoperative period, the rate of decline in serum bilirubin levels directly correlates with a positive prognosis.

  • One study evaluated 244 infants who were enrolled in the prospective Childhood Liver Disease Research and Education Network and underwent Kasai portoenterostomy (KPE). The results noted that at 1 and 2 years post-KPE, the transplant-free survival rate was 53.7% and 46.7%, respectively. Risk of transplant/death was significantly lower in patients who achieved bile drainage within 3 months post-KPE, while it increased in patients with porta hepatis atresia, nonpatent common bile duct, biliary atresia splenic malformation syndrome, nodular liver appearance compared with firm, and age at KPE. No association with outcome was noted with gestational age, sex, race, ethnicity, or extent of porta hepatis dissection. [20]

  • As discussed in Mortality/Morbidity, bile flow, even if achieved at surgery, may be inadequate in as many as one third of patients after the initial postoperative period. These children require early (< 2 y) liver transplantation. Practice guidelines for the evaluation of a patient for liver transplantation have been established by the American Association for the Study of Liver Diseases. [21] Factors that predict improved long-term outcome after Kasai portoenterostomy include the following:

    • Younger than 8 weeks at operation

    • Preoperative histology and ductal remnant size

    • Presence of bile in hepatic lobular zone 1

    • Absence of portal hypertension, cirrhosis, and associated anomalies

    • Experience of the surgical team

    • Postoperative clearing of jaundice

  • The following 3 categories of patients with extrahepatic biliary atresia should be considered for reexploration following a Kasai or modified Kasai portoenterostomy:

    • Infants who become jaundiced after an initial anicteric phase postoperatively

    • Infants with favorable hepatic and biliary duct remnant histology at initial operation, who do not successfully drain bile

    • Infants who may have had an inadequate initial surgery

  • Extrahepatic biliary atresia is the most common primary diagnosis in children requiring orthotopic liver transplantation (OLT), comprising more than 50% of patients with liver transplants in most series. [22]

    • Overall, a review demonstrated that 66% of infants undergoing the Kasai procedure ultimately required OLT, including more than 50% of patients who initially achieved bile drainage.

    • In most series reported to date, the primary indications for OLT are the symptoms of end-stage liver disease and/or hepatic failure, including progressive cholestasis, recurrent cholangitis, poorly controlled portal hypertension, intractable ascites, decreased hepatic synthetic function (eg, hypoalbuminemia, coagulopathy unresponsive to vitamin K), and growth failure.

    • As long-term outcomes following OLT in children continue to improve (along with increased living donor availability) using split-liver grafts, application of this surgical modality for early treatment of biliary atresia will likely increase, certainly in patients with inadequate bile flow following portoenterostomy.

  • A study by LeeVan et al that included 626 children with biliary atresia reported that even though those that underwent primary liver transplant had a higher mortality rate after 3 months, those who underwent primary liver transplant had a reduced risk of long-term mortality than those managed with biliary-enteric drainage treatment after 6 months. [23]
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