Biliary Atresia (BA) is the commonest neonatal liver disease in New Zealand affecting 1 in

8000 live births, with increased frequency in Maori and Pacific children (approximately 1 in
5000). It is the most common indication for liver transplantation in childhood.

Features of BA include conjugated jaundice, pale stools and dark urine. The treatment is
surgical (Kasai portoenterostomy) which is most successful if performed early, preferably
before 6 weeks of age. Approximately 60% of infants with BA will have a successful Kasai
(bilirubin <25mmol/L by 6 months of age). 

Infants with BA who have unsuccessful Kasai will require transplantation. Those with
successful Kasai may still require transplantation due to portal hypertension, recurrent
cholangitis and complications of cirrhosis. 

The increased frequency in NZ and the tight timeframe in which to perform surgery means
the emphasis is on early referral to a paediatric gastroenterology service and prompt
diagnosis.

Please note:

 The need for isotope excretion scan has been removed in this version of the
guideline given it lacks specificity to distinguish biliary atresia from any other form of
cholestatic liver disease and has the potential to delay diagnosis

 Exclusion of alpha-1-antitrypsin deficiency by blood testing has also been removed

due to low yield and potential to delay diagnosis of biliary atresia while awaiting results.

Diagnosis

History and examination

Cardinal features include:

 jaundice

 pale stools, becoming paler once meconium clears

 dark urine

 stool colour should be directly observed by a healthcare professional

( [Link]

There should be an absence of features which may indicate other neonatal liver
disease, such as:

 Congenital heart disease

 Facial features of Alagille syndrome

 Family history of genetic liver disease

 Alpha-1-antitrypsin phenotype should be requested but the result is not critical prior
to operative cholangiogram and Kasai in babies whose ultrasound and liver biopsy are
highly suggestive of BA

 Alpha-1-antitrypsin level is not recommended as it is an acute phase marker and

cannot accurately differentiate between carriers and affected individuals

Syndromic biliary atresia (biliary atresia-splenic malformation)

10% cases have extra-hepatic features which may include:

 Abdominal situs inversus

 Intestinal malrotation

 Polysplenia (often poorly functional)

 Pre-duodenal portal vein

 Portal vein and IVC anomalies

 Congenital heart disease

These children have unique considerations if liver transplantation is required

Investigations
Any infant with jaundice beyond two weeks of age or pale stools should undergo clinical
assessment and have blood sent for a split bilirubin. If conjugated hyperbilirubinaemia (>20
mmol/L or >20% total bilirubin) is confirmed, perform first line investigations as per Starship
Clinical Guidelines.

Laboratory features

 Conjugated hyperbilirubinaemia (>20mmol/L or >20% total bilirubin)

 Mild-moderate elevation of AST, ALT, GGT, ALP

 INR or prothrombin time should be measured as infants may be vitamin K deficient

 Fat soluble vitamin deficiencies (vitamins A, D, E, K) are common

Radiological features

 Absent, small or contracted gall bladder on fasting abdominal ultrasound - note the
presence of a gall bladder does not exclude BA
 Isotope excretion scan is not recommended as it delays diagnosis due to need for
phenobarbitone priming. It can however be reserved for investigation of jaundice in selected
infants with ambiguous results, on request of the hepatologist

Histological findings on percutaneous liver biopsy

 Cholestasis

 Fibrosis

 Proliferation of bile ductules

 Absence of diagnostic features of other neonatal liver diseases

 Liver biopsy should be assessed by a paediatric pathologist experienced in the

diagnosis of BA

 Features may become more obvious with time so infants with jaundice and pale stool
whose initial biopsy is not diagnostic may require a second biopsy if signs do not improve

Management - pre-operative to 6 months following Kasai

Operative management should be undertaken by a paediatric surgeon experienced in the
management of biliary atresia, who performs cholangiograms and Kasai portoenterostomy
on a regular basis.

A cholangiogram that shows a normal intra hepatic biliary tree, normal external hepatic
ducts and normal drainage into the duodenum excludes the diagnosis.

If biliary atresia is confirmed at exploration, the child will proceed to Kasai portoenterostomy
at the same operation.

Pre-operative management

INVESTIGATIONS

Initial investigations as per prolonged jaundice guideline determine whether to proceed to

operative cholangiogram +/- Kasai portoenterostomy

Day before Kasai

• FBC                                               • Clotting profile
• U&E                                                • Group and hold
• LFTs                                              • Chest xray as clinically indicated
 Nutritional management

 All babies with conjugated jaundice will be seen by a dietitian with experience in
managing paediatric liver disease

 For formula-fed infants, consideration will be given to switching to a feed with

medium chain triglyceride as the predominant lipid source eg PeptiJunior

 For breast-fed infants, consideration will be given to introducing some supplemental

formula feeds using a medium chain triglyceride based feed eg Pepti-Junior while allowing
the baby to continue some breast feeding. Another option would be to add MCT to
expressed breast milk

Vitamin supplementation

 Children with BA are almost always deficient in fat-soluble vitamins A, D, E and K

 Oral supplements should be commenced as soon as conjugated jaundice is detected

and should continue throughout - see Starship Clinical Guideline on Prolonged Jaundice

 Baseline levels of vitamins A, D and E should be requested but it is not necessary to

await results before commencing supplements

 If INR or prothrombin ratio is abnormal, IV vitamin K should be prescribed and

continued daily until INR or PR is normal, then switched to oral

 Vitamin doses should be titrated according to growth and vitamin levels 

See Vitamin Supplementation Table for dosing information

Parental education and support

 All families whose babies are suspected of having BA will have access to a Clinical
Nurse Specialist (CNS) who can provide education about BA and liver disease

 Families should be offered a referral to a social worker and the Consult Liaison
Team for psychological support

 Other referrals which may be considered include, to a lactation consultant and

maternal mental health on the guidance of the Consult Liaison Team

Intra-operative management
Anaesthetic considerations

 Percutaneous central line will usually be placed. As far as possible, the right sided
neck veins should be avoided as these may be required during subsequent liver
transplantation. Urinary catheterisation will be required
 Consideration will be given to epidural analgesia

Surgical approach

 Surgery should be carried out in a centre with a multidisciplinary team experienced in

the care of paediatric liver disease

 A paediatric surgeon with specific expertise in Kasai portoenterostomy will undertake

the surgery

 The surgeon will directly note the upper gastrointestinal anatomy along with the
appearance of the liver and extra-hepatic biliary tree and may perform an intra-operative
cholangiogram to confirm the findings

 An intraoperative cholangiogram should not be performed as a diagnostic test in

isolation, but should be performed with a view to proceeding to Kasai, should the diagnosis
of BA be confirmed.

 The type of biliary atresia will be noted for future reference

 A standard, open Kasai portoenterostomy is the preferred operation

 If evidence of severe cirrhosis with portal hypertension, the surgeon may elect not to
proceed with Kasai, in view of the inevitability of progression of liver disease. These children
should receive intensive nutritional support and should be assessed for transplant without
undue delay.

Antimicrobial cover

 IV cefazolin at induction at 30/mg/kg/dose to continue 6 hourly post-operatively

for 72 hours

 If operating time is > 4 hours or blood loss > 50% total volume re-dosing should
occur at 4 hours

Intravenous fluids

 Intravenous fluid management should be prescribed following the Starship

Intravenous Fluids Clinical Guideline.

Post-operative management during initial hospital stay

Return to the ward

 Most babies can be cared for in the paediatric surgical HDU on ward 24B

 Initial management will be undertaken by the Paediatric Surgical team who will
transfer the baby to the Paediatric Hepatology (Gastroenterology) team once the baby is
stable
 Antimicrobial cover

 IV cefazolin 30/mg/kg/dose to continue 6 hourly post-operatively for 72 hours or until

the baby is feeding and can tolerate oral antibiotics

 On completion of IV antibiotics commence long term oral cholangitis prophylaxis with

co-trimoxazole (2mg/kg/day as trimethoprim component once daily) for 12 months post
Kasai

 For children who experience suspected or established cholangitis, co-trimoxazole

will be continued until 12 months following the most recent cholangitis episode

 Ensure co-trimoxazole dose is regularly adjusted for weight gain

Steroid therapy

 Start steroids on day 1 post op unless there is fever or evidence of sepsis:

Methylprednisolone IV 20mg daily decreasing by 2.5mg daily until 5mg/day
Then prednisolone 5mg daily orally for one further week then stop

 Babies who are otherwise ready for discharge can be transitioned to oral steroids
earlier than 7 days

 Evidence for the benefit of steroid therapy on the long-term outcome of BA is weak
and will be reviewed every 2 years

Fat soluble vitamin supplementation

 Oral fat soluble vitamin supplements will be re-commenced as soon as practical

following surgery
Vitamin K:
Day 1: Start 2mg IV daily for 3 days
Day 4: Switch to enteral dose as per vitamin supplementation table
If there is a delay to feeding or INR/prothrombin ratio is prolonged, continue IV vitamin K
until the baby is feeding and INR/prothrombin ratio is normal

Analgesia

 Analgesia will be provided by oral/enteral (preferred) or IV paracetamol and either an

epidural or IV NCA (Nurse Controlled Analgesia) morphine.

 Choice of analgesia on return to the ward is at the discretion of the anaesthetist and
dependent on the clinical status of the child

 Epidural or NCA will be continued until the child is tolerating feeds and then they will
be transitioned to oral medication for 24-48 hours

 Paracetamol dosing will be 10 mg/kg/dose up to 4 times daily. In general, non-

steroidal anti-inflammatory drugs should be avoided in children with liver disease
 Fluids

 Day 1: 70% maintenance fluids as per Starship Intravenous Fluids Clinical

Guideline - if able, to be administered as 50:50 IVF and Pedialyte

 Day 2: Change Pedialyte to continuous enteral milk feeding via NG tube at

50ml/kg/day in addition to the IV fluids. The type of milk feed will be directed by the
paediatric dietitian

 Day 3: Commence bolus feeds, the volume and type being directed by the surgical
team and paediatric dietitian. IV fluids will be weaned accordingly

 Day 4 onwards will depend on progress over the previous days

Nutrition

 See fluids section above for timing of feed commencement post-operatively

 As pre-operatively, the choice of feed depends on whether the baby is predominantly

breast or formula fed

 For formula-fed infants, a feed with medium chain triglyceride as the predominant
lipid source eg PeptiJunior will be used

 For breast-fed infants, consideration will be given to using some supplemental

formula feeds with a medium chain triglyceride based feed eg Pepti-Junior while allowing
the baby to continue some breast feeding. Another option would be to add MCT to
expressed breast milk

 Supplementation with medium chain triglyceride based feeds will be continued at

least until it is known whether the Kasai has been successful

 Due to the importance of good nutrition in managing paediatric liver disease, there
will be a low threshold to commence nasogastric tube feeding in the event of sub-optimal
weight gain

Monitoring

 Stool colour will be recorded daily and in particular whether pigment appears in the
stool. One stool sample daily should be collected into a stool pot for review by the medical
team

 Day 1: FBC, LFTs, coagulation screen, electrolytes

 Thereafter, blood tests will be undertaken as clinically indicated but usually at least
twice a week in the first week

 Urinary catheter will be removed as soon as urinary output is stable (aim

≥1mlkg/kg/hour) and will be at the direction of the Paediatric Surgeon (usually day 2-3)
 Prior to discharge from hospital
The Clinical Nurse Specialist (CNS) will provide further education to the family prior to
discharge and ensure family are aware of follow up arrangements in the community. The
family will be provided with;

 Discharge letter

 Blood forms for ongoing monitoring

 The paediatric liver disease - Biliary Atresia: a parent guide

  Kids Foundation welcome pack with application for membership

 The CNS will ensure any referrals to home care nursing teams = (for dressing
changes or monitoring of weight) after discharge are completed

 The CNS will ensure that the family and GP are aware of the plan for the baby's
immunisations which may have been delayed due to medical reasons eg prednisone

 The CNS will provide a copy of the accelerated immunisation schedule to the family
and the GP service

 For babies who live outside Auckland, the CNS will contact the local paediatric team
with details of the follow-up plan, including blood monitoring and clinic arrangements the
accelerated immunisation schedule and the link to the shared care nursing resource 

 A prescription for discharge medications will be provided

 Repeat referrals can be made to social work and the Consult Liaison Team if
required

Post-operative management following discharge (up to 6 months

following Kasai)
Blood monitoring

 At least weekly LFTs for the first month following Kasai, spacing out to fortnightly and
monthly thereafter for the first 6 months

 Monthly coagulation, FBC, U&E, vitamins A, D, E and other investigations as

required

 For children outside Auckland and Christchurch, results should be copied

(electronically if possible) through to the local paediatric hepatology service

Other monitoring
 Weekly weights for the first month, preferably by a home care nursing service using
the same scales spacing out to fortnightly then monthly for the first 6 months

 Sub-optimal growth according to the WHO infant growth chart should be reported to
the Auckland or Christchurch paediatric hepatology service

 Need for abdominal ultrasound will be determined by progress and will be advised by
the Auckland or Christchurch paediatric hepatology service

Clinic appointments

 Frequency will depend on where the baby lives and their progress

 Auckland and Christchurch patients will generally remain under the sole care of the
paediatric hepatology service unless there are other indications for referral to general
paediatrics

 Other patients will predominantly be managed in a shared care model between the
Auckland or Christchurch paediatric hepatology service and a local general paediatrician

 As far as possible, infants living outside Auckland and Christchurch will be seen in
Paediatric Gastroenterology visiting outreach clinics if these exist locally

 Initially, babies will be reviewed locally a week following discharge, spacing out to
fortnightly then a minimum of monthly appointments

 At a minimum, the paediatric hepatology service will review the babies at 1, 3 and 6
months following Kasai to determine the success or otherwise of the operation and to plan
future care

 Reasons for more frequent review include, but are not limited to:
- Poor growth
- Synthetic liver dysfunction
- Ascites
- Portal hypertension and/or gastrointestinal bleeding
- Recurrent cholangitis
- Clear need to proceed to liver transplant assessment

Possible complications

 Cholangitis

 Portal hypertension

 Poor nutritional management