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Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review
Teru Kumagi1, Joost P H Drenth, Orlee Guttman
1Toronto Western Hospital, Toronto, Canada.
Insights
Biliary atresia survivors often have native liver health into adulthood, but commonly develop secondary biliary diseases like cholangitis and portal hypertension. This review examines their long-term outcomes.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia is a severe, infant-only progressive biliary injury.
- Understanding long-term outcomes is crucial for managing affected individuals.
Purpose of the Study:
- To review the outcomes of patients with biliary atresia who survive into adulthood without requiring a liver transplant in childhood.
- To identify common complications and long-term health issues in this cohort.
Main Methods:
- A multicenter review was conducted.
- Patients included those with biliary atresia treated surgically who reached adulthood without transplantation.
- Data on clinical features, liver enzymes, bilirubin, complications, and transplant history were analyzed.
Main Results:
- Twenty-two adult patients with biliary atresia were identified.
- Most (95.5%) were alive, with 81.8% retaining their native liver.
- Common findings included portal hypertension (95.5%), elevated liver enzymes, pruritus (40%), jaundice (50%), and features of sclerosing cholangitis (59.1%).
- Half (50%) had a history of bacterial cholangitis.
Conclusions:
- Some patients treated for biliary atresia can survive into adulthood with their native liver.
- Secondary biliary disease, including cholangitis and portal hypertension, is common in these survivors.
- Long-term native liver survival is possible but often associated with significant biliary complications.
Background:
Biliary atresia is a progressive biliary injury which occurs only in infants.
Aims:
To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.
Methods:
A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.
Results:
Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.
Conclusions:
Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.
