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Biliary Atresia - guidelines for diagnosis and management

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The Diagnosis and Management of Biliary Atresia (BA)

The National Paediatric Gastroenterology Clinical Network has agreed that ALL infants with conjugated hyperbilirubinaemia should be referred to a Paediatric Gastroenterologist or Hepatologist for initial investigation and subsequent management. For North Island patients, the Paediatric Gastroenterology and Hepatology service is based at Starship Child Health, Auckland and for South Island patients, the Paediatric Gastroenterology and Hepatology service is based at Christchurch Hospital. The Gastroenterology or Hepatology team will ensure that all investigations have been completed and other liver diseases considered before the child is considered for laparotomy and possible Kasai portoenterostomy

 

In This Document:

Background
Diagnosis 
Management - pre-operative to 6 months following Kasai 
Management after 6 months post Kasai 
Complications of biliary atresia (including cholangitis and portal hypertension) 
Nutritional management of biliary atresia 
Fat soluble vitamin supplementation 
Immunisations 
Screening for hepatocellular carcinoma (HCC) 
Indications for liver transplantation

Background

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:

Diagnosis

History and Examination

Cardinal features include:

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

Syndromic biliary atresia (biliary atresia-splenic malformation)

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

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

Radiological features

Histological findings on percutaneous liver biopsy

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                                              
• U&E                                               
• LFTs                                             

• Clotting profile
• Group and hold
• Chest xray as clinically indicated
NUTRITIONAL MANAGEMENT
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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
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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
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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
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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
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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
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• 
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
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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
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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
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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
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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
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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
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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
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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
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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;

Post-operative management following discharge (up to 6 months following Kasai)

BLOOD MONITORING
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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
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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
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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
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Cholangitis
Portal hypertension
Poor nutritional management

Management after 6 months post Kasai

Management of infants with successful Kasai (normal bilirubin at 6 months following Kasai)

BLOOD MONITORING
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3 monthly coagulation, FBC, U&E spacing out to 6 monthly in older children
Vitamins A, D, E until levels are normal then annually thereafter
Annual: vitamins A, D, E, AFP, hepatitis A and B serology
For children outside Auckland and Christchurch, results should be copied through to the Auckland or Christchurch paediatric hepatology service
OTHER MONITORING

 


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Monthly weights for the first year, preferably by a home care nursing service using the same scales after which growth can be assessed in clinic appointments
Sub-optimal growth according to the WHO infant growth chart should be reported to the Auckland or Christchurch paediatric hepatology service
Abdominal ultrasound should be performed annually with specific attention to:
- Spleen size
- Evidence of cirrhosis and portal hypertension
- Dilated bile ducts or bile lakes
- Focal liver lesions
The paediatric hepatology service will advise whether the USS needs to include Doppler assessment of the hepatic vasculature
For children who are old enough to breath hold on request, elastography (or FibroScan) if available, can be a useful tool to look for development of fibrosis
Radiology investigations can usually be performed locally and referred to the paediatric hepatology service via the PACS radiology system
NUTRITION
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Infants whose Kasai has been successful can usually switch to a conventional infant formula once their bilirubin is normal
Solids foods should be introduced at the usual time
Older children with a successful Kasai can eat a normal diet
Children with recurrence of jaundice will likely need liver transplant assessment and nutritional intervention as per the liver transplant protocol
FAT SOLUBLE VITAMIN SUPPLEMENTATION
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Fat soluble vitamin supplements can usually be discontinued once the bilirubin has been normal for one month and serum vitamin levels are normal
Vitamin levels should be monitored at least annually and supplements re-introduced if needed
CLINIC APPOINTMENTS

 


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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, children living outside Auckland and Christchurch will be seen in Paediatric Gastroenterology visiting outreach clinics if these exist locally
At a minimum, the paediatric hepatology service will review patients at 6 monthly intervals and can alternate visits with the local paediatrician
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
TRANSITION TO ADULT SERVICES
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Young people will be transitioned to adult services via the Young Persons Liver Clinic held jointly with the adult hepatology service at Auckland City Hospital as they may still require liver transplantation at a later stage. Those living outside Auckland will also require a local transition to adult gastroenterology services
General lifestyle advice will be offered at this stage
Young people with BA will require re-education about their disease and the long-term implications

Complications of biliary atresia (BA)

Cholangitis

Background

Investigations

Treatment

FIRST LINE THERAPY
  Cefotaxime
Amoxicillin
50mg/kg q8 hourly and
50mg/kg q8 hourly IV
OR Gentamycin
Amoxicillin
7.5mg/kg/dose <10 years, 5mg/kg>10 years
50mg/kg q8 hourly IV
SECOND LINE THERAPY
  Gentamycin
Amoxicillin
7.5mg/kg/dose <10 years, 5mg/kg>10 years
50mg/kg q8 hourly IV
OR Ceftazidime
Amoxicillin
50mg/kg q8 hourly and
50mg/kg q8 hourly IV
OR Tazocin
Amoxicillin
90mg/kg q8hourly ( max 4.5gms) and
50mg/kg q8 hourly IV

Cholangitis prophylaxis


Infectious diseases approval for antibiotics

Portal hypertension

Portal hypertension is one of the commonest long-term complications of BA and an indication for liver transplant even if Kasai has been successful.  There are no good non-invasive tools to screen for portal hypertension but clues may include:

Features which suggest varices should be treated include:

Gastrointestinal (GI) bleeding should be managed urgently via the GI bleeding protocol and likely indicates a need for liver transplant assessment. Varices per se may not require transplantation if small, not rapidly progressive and without any GI bleeding. However, all varices and portal hypertension should be managed by the paediatric hepatology service who can decide on the need for transplantation

Nutritional management in biliary atresia

Fat-soluble vitamin supplementation

Vitamin Supplementation Dosage and Administration
Vitamin A:
Available preparation: Vitadol C® = 2000 micrograms vitamin A per gram = 1 ml =7500IU
Starting dose is 1ml once daily, then titrate dose based on 3 monthly vitamin levels.

Vitadol C® is fully subsidised in the community.
Vitamin D:
Starting dose: 30-50 nanograms/kg once a day rounded to nearest 100 nanograms, then titrate dose based on 3 monthly vitamin levels.

To be prescribed by dose volume, NOT number of drops, to the nearest 100ng to avoid inaccurate dosing associated with dropper choice.

Available preparation: Alfacalcidol (One-Alpha drops®)
Vitamin E:
Starting dose: 50 mg (68 IU) once a day
Available preparation: = d-alpha-tocopheryl acetate (Micel* E®) = 156 IU/ml

Suggested starting dose 0.5 ml once daily, then titrate dose based on 3 monthly vitamin levels.Requires PHARMACapplication for funding - refer to PHARMAC website https://www.pharmac.govt.nz/pharmacEforms/nppaupload.php
Vitamin K:
Fat soluble vitamin deficiency secondary to cholestasis

Phytomenadione (Konakion ®): Dose 2mg to 10mg oral or IV. Starting dose is 2mg daily which can be increased according to INR

Konakion is only available as an IV preparation which can be used for oral dosing.Available as 2mg and 10mg ampoules, round dose accordingly to achieve easy to draw up volumes. NB: The 2 mg amps are provided with a draw up syringe so please identify this product when prescribing

Immunisations

Screening for hepatocellular carcinoma (HCC)

Indications for liver transplantation

References

Document last reviewed: April 2017

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