Approach

Once the diagnosis is confirmed, surgical intervention is recommended as soon as possible, ideally by 30-45 days of life.[49][50][51][52]​​​​​​ Patients who have surgery after 90 days have worse outcomes (as measured by the need for liver transplantation or death within the first 10 years).[49][75]​​ The recommended initial treatment for infants without end-stage liver disease is hepatoportoenterostomy, although ultimately up to 80% require liver transplantation.[76]

Hepatoportoenterostomy (HPE)

The procedure involves ligating the fibrous duct above the join with the duodenum and dissecting proximally to the porta hepatis, from which bile usually flows from the liver. A piece of small intestine is attached to this point, allowing direct drainage. Successful surgery allows some bile drainage and prevents or delays liver damage. If total bilirubin is less than 34.2 micromoles/L (2 mg/dL) at 3 months post-HPE, then the chance of being transplant-free at 2 years of age is 84%.[77] Revision procedures are not recommended, as these can be very difficult and complications may prevent liver transplantation.

Liver transplantation

Liver transplantation is reserved for those in whom HPE is unsuccessful or those who have extensive liver damage at the time of diagnosis.[78]​ The National Institutes of Health consensus conference on liver transplantation recommended that transplant should be delayed as long as possible to allow for maximal growth of the child. End-stage liver disease is recognised by progressive cholestasis, hepatocellular decompensation, or the development of severe portal hypertension.[79] Outcomes for paediatric patients undergoing primary liver transplantation for biliary atresia are favourable, with 1-year patient and graft survival rates of 92.1% and 83.6%, respectively.[80] Biliary atresia patients can receive whole and split cadaveric livers, as well as segments from living donors.

Management of complications post hepatoportoenterostomy

The most common complications or sequelae are growth failure, portal hypertension, cholangitis, and ascites.[78][81]​ Long-term management focuses on optimisation of growth and nutrition.

Ursodeoxycholic acid promotes bile flow.[82]​ It can be given to all patients as soon as urinary bile acids have been sent for analysis, as long as their total bilirubin is less than 256.6 micromoles/L (15 mg/dL). It is continued until resolution of the jaundice and use after this is physician-dependent. Treatment can be lifelong. 

Nutrition

During the first year, either fortified breast milk or medium-chain triglyceride-enriched formula is given with monthly monitoring of growth and nutritional status.[78][83]​​​ After 1 year, if growth is normal, it is possible to switch to a regular diet or supplement diet with enriching medium-chain triglycerides in powder form that can be added to food. 

Fat-soluble vitamins are given to all children with biliary atresia.[78][82]​​​​ Levels are monitored monthly, and doses are adjusted accordingly. If growth develops normally after 1 year, the child may be switched to multivitamins and annual follow-up. 

Antibiotic prophylaxis

All patients receive antibiotic prophylaxis for the first year of life to prevent cholangitis.[84][85]​​ After 1 year, the decision to continue antibiotic prophylaxis is physician-dependent and made on a case-by-case basis. The usual antibiotics used are trimethoprim/sulfamethoxazole, but neomycin can be given if the patient is allergic to this combination.[78]​

Management of patients post liver transplant

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.

Use of this content is subject to our disclaimer