Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

infants with biliary obstruction without end-stage liver disease

1st line – hepatoportoenterostomy (HPE)

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ACUTE
|
infants with biliary obstruction without end-stage liver disease
1st line – 

hepatoportoenterostomy (HPE)

HPE should ideally be performed before 45 to 60 days of life, to alleviate obstruction and prevent liver damage.[65][66] If total bilirubin is <2 mg/dL at 3 months post-HPE, then the chance of being transplant-free at 2 years of age is 84%.​[67]​

Consider – ursodiol

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ACUTE
|
infants with biliary obstruction without end-stage liver disease
Consider – 

ursodiol

Treatment recommended for SOME patients in selected patient group

Thought to be hepatoprotective and facilitates bile flow.

Can be started after urinary bile acids have been sent for analysis, and continued until resolution of jaundice, after which continuation is physician-dependent.

If the total bilirubin is >15 mg/dL then it should not be given, as the bile acid load is too high and ursodiol is unlikely to be helpful.

Primary options

ursodiol: 20-30 mg/kg/day orally given in 2 divided doses

2nd line – liver transplantation

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ACUTE
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infants with biliary obstruction without end-stage liver disease
2nd line – 

liver transplantation

If hepatoportoenterostomy is unsuccessful, liver transplantation would be the only treatment. It is considered in patients with signs of end-stage liver disease, progressive cholestasis, hepatocellular decompensation, or the development of severe portal hypertension. [69]

The management of patients after liver transplant should be handled by a specialist with expertise in managing transplant patients, and is beyond the scope of this topic.

Consider – ursodiol

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ACUTE
|
infants with biliary obstruction without end-stage liver disease
Consider – 

ursodiol

Treatment recommended for SOME patients in selected patient group

Thought to be hepatoprotective and facilitates bile flow.

Can be started after urinary bile acids have been sent for analysis and continued until resolution of jaundice, after which continuation is physician-dependent.

If the total bilirubin is >15 mg/dL then it should not be given, as the bile acid load is too high and ursodiol is unlikely to be helpful.

Primary options

ursodiol: 20-30 mg/kg/day orally given in 2 divided doses

infants with biliary obstruction with end-stage liver disease

1st line – liver transplantation

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ACUTE
|
infants with biliary obstruction with end-stage liver disease
1st line – 

liver transplantation

If patients are presenting with signs of end-stage liver disease, such as frank ascites and variceal bleeding, then liver transplantation is the only option. Without surgery, the condition would ultimately be fatal.

ONGOING

post hepatoportoenterostomy

1st line – antibiotic prophylaxis

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ONGOING
|
post hepatoportoenterostomy
1st line – 

antibiotic prophylaxis

All patients receive antibiotic prophylaxis for the first year of life. After 1 year, the decision to continue antibiotic prophylaxis is physician-dependent and made on a case-by-case basis.

The usual choice is trimethoprim/sulfamethoxazole. It is contraindicated in children <2 months of age due to the risk of kernicterus, but is still used with caution. Neomycin can be used if the patient is allergic to trimethoprim/sulfamethoxazole.

Primary options

sulfamethoxazole/trimethoprim: 4 mg/kg/day orally given in divided doses every 12 hours

More

Secondary options

neomycin sulfate: 25 mg/kg/day orally given in divided doses every 8 hours

More

Consider – ursodiol

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ONGOING
|
post hepatoportoenterostomy
Consider – 

ursodiol

Treatment recommended for SOME patients in selected patient group

Thought to be hepatoprotective and facilitates bile flow.

Can be started after urinary bile acids have been sent for analysis and continued until resolution of jaundice, after which continuation is physician-dependent.

If the total bilirubin is >15 mg/dL then it should not be given, as the bile acid load is too high and ursodiol is unlikely to be helpful.

Primary options

ursodiol: 20-30 mg/kg/day orally given in 2 divided doses

Plus – nutritional support and vitamin supplementation

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ONGOING
|
post hepatoportoenterostomy
Plus – 

nutritional support and vitamin supplementation

Treatment recommended for ALL patients in selected patient group

Used to promote growth and nutrition.

Fortified breast milk or medium-chain triglyceride-enriched formula is given, with monthly monitoring. Higher concentrations of formula can be used if required to further promote growth. After 1 year, if growth is normal, it is possible to switch to a regular diet or supplement diet with enriching medium-chain triglycerides, a powder that can be added to food.

Fat-soluble vitamins are given to all children with biliary atresia. Levels are monitored monthly and doses adjusted accordingly. If growth develops normally, the child may be switched to multivitamins and annual follow-up after 1 year.

Proprietary combination formulations of vitamin A, D, E, and K are available to improve patient compliance.

Primary options

vitamin A (retinol): 3000 units orally once daily for 1 year

and

alpha-tocopherol (vitamin E): 15-25 units/kg orally once daily for 1 year

and

ergocalciferol (vitamin D2): 4000 units orally once daily for 1 year

and

phytonadione (vitamin K1): 2.5 mg orally three times weekly for 1 year

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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