What is Biliary Atresia (BA)?

Bile is a digestive juice secreted from the liver. Bile passes into the intestine through a tube like structure which is called bile duct. Bile juice is important for the digestion of fat in food and for absorption of fat soluble vitamins. Poor development of the bile duct is called Biliary Atresia. This can lead to cholestatic (direct) jaundice or liver damage.

How common is Biliary Atresia?

Biliary Atresia occurs in 1 in 10,000 to 20,000 live births. It is one of the commonest causes of neonatal jaundice.

What is the cause of Biliary Atresia?

The exact cause is not known. Various researches have suggested that there can be either viral, toxin or genetically related damage to the biliary pathways.

Is Biliary Atresia an inheritable disease?

It is not an inheritable condition. So if one baby suffers from Biliary Atresia then there is very small chance that the other sib may also suffer from this same disease.

How will I be able to identify this disease?

Babies with Biliary Atresia are normal at birth. They tend to grow well initially. Jaundice is the first sign. It may appear from birth to eight weeks of age. It is noticed when stool appears pale or white (clay colored) as there is no bile pigment. Urine is dark yellow (mustard oil) and stain the diaper or nappy yellow (newborn urine is colorless water like till 3 months of age). In later stages abdomen looks full due to increased size of liver and spleen. Occasionally bleeding can occur from the nose or umbilical cord.

How will the doctor diagnose this condition?

Biliary Atresia is suspected when there is increase in the blood level of direct bilirubin with little increase in liver enzymes (SGPT, SGOT). One liver enzyme which is markedly raised is GGTP. It is usually a combination of 3 tests which confirm the diagnosis.

• USG abdomen shows that gall bladder is either absent or abnormal shape or small size. Common bile duct is not seen.

• HIDA scan: This is a nuclear imaging scan. This is done by injecting a dye. Normal liver will take up the dye and excrete into the intestine. In Biliary Atresia, liver uptake is usually normal but the dye does not pass into the intestine. Before doing this test some doctors may prescribe 5 days of syrup phenobarbitone to improve the test efficacy. However, in many other conditions, the dye will not pass in to the intestine. If HIDA scan is normal, we can rule out Biliary Atresia

• Liver biopsy: liver biopsy may be done before going for surgical intervention or it can be done during surgery. It needs an experienced pathologist to confidently diagnose Biliary Atresia.

Is surgery always indicated?

Biliary Atresia is a surgical illness and Kasai procedure (named after Japanese surgeon who invented it) is the treatment of choice. Best results are seen if the surgery is performed within 60 days of birth.

How is the surgery performed?

The surgery is done in two steps. In first step the diagnosis of Biliary Atresia is once again confirmed by putting a contrast dye in the visible bile duct and X-ray is taken to see if the dye reaches the intestine. If the dye is not seen in the intestine the diagnosis is confirmed. In the second step the surgeon performs the corrective surgery. In this procedure the surgeon cuts the area where the bile duct joins the liver (hilum) and joins it to the side of the intestine by taking sutures. The rationale of this surgery is based on the assumption that some small ducts are patent at the hilum of the liver which will directly drain into the intestine.

What care should be taken post procedure?

After the procedure the baby is shifted to pediatric intensive care unit. He will be given antibiotics, IV fluids and will be kept nil by mouth. The mother should keep expressing her breast milk. After 2 days the doctor will introduce expressed mother milk through a feeding tube and monitor his abdomen. Once he tolerates feeds and is stable, he will be shifted to wards. Within a week period baby will get discharged from the hospital. At discharge the doctor will start the supportive management of jaundice.

• Nutrition: Due to poor absorption of fat by the intestine, the caloric requirement is 1.5 times the normal requirement. To increase the calorie intake, Simyl MCT oil can be added to the milk. The protein intake should be 3-4 gm/kg/day. Fat soluble vitamins like A, D E and K need to be supplemented. B complex vitamins need to be given 2-3 times the recommended daily allowance

• Prevention and management of infections: antibiotics
• Choleretics: These are the drug which makes the bile to flow easily. UDCA is used in the dose of 15-30 mg/kg/day in 2 divided doses.
• Regular follow up and monitoring
• Vaccination as per schedule (take precaution during injection in muscle, inform the doctor/nurse regarding bleeding tendency, press for 5 min, do not rub)

What is the success rate of this procedure?

If the surgery is done within 6 weeks then the success rate is 80-90%. If done between 6 weeks to 12 weeks then the success rate will vary from 50-80%. Beyond 12 weeks the success rate is minimal. The success in Kasai procedure means that the bile drainage is established. But despite adequate drainage progressive slow liver damage continues. Many of the patients will ultimately require liver transplantation.

According to a study, if surgery is done within 30 days the chance of survival with patients own liver is 50% at 4 years. Between 30-90 days, the chance of survival is 36% at 4 years. If surgery is done after 90 days then most of the patients will require liver transplant within first year of life.

How will I know whether the surgery is successful?

When the surgery is successful, you will notice that within few weeks the baby stool becomes yellow, the urine becomes less yellow and the jaundice of skin and eyes starts decreasing. The doctor will repeat tests after 2-3 weeks which will show decreasing trend of blood bilirubin.

If many of the patients require liver transplantation (LT), then why Kasai procedure is necessary?

This initial surgery prevents the immediate need of LT in such a small baby. It allows the baby to grow and lead a near normal life. In some cases the child may not require LT even till adulthood. The success rate of liver transplant is much higher with comparative lesser side effects when done in older individual.

What are the complications one should except in first year of life?

The baby may develop infection of the bile pathway within the liver as the intestine is attached to it. This is called ascending cholangitis. The baby develops fever, irritability due to abdominal pain and vomiting. Oral or IV antibiotics is the treatment of choice.

If the surgery is unsuccessful, the baby may develop features of liver failure. There is increase in jaundice, gradual enlargement of abdomen, blood in vomits, black stools (Malena) or fluid in abdomen (Ascites).

What are the indications of liver transplantation?

Consultation with the expert pediatric gastroenterologist is a must. The indications of LT are:

• Primary failure (lack of bile drainage) of the Kasai procedure.
• Poor growth despite all measures : This is common in children with Cholestasis
• Complications of portal hypertension not responding to endoscopic management. (blood in vomits, malena, enlargement of spleen)
• Repeated upper intestinal bleeding
• Refractory Ascites (fluid in abdomen) that compromises lung, bowel, or kidney function
• Complications of lung (Hepatopulmonary syndrome)
• Progressive liver dysfunction
• Progressive Cholestasis
• Refractory coagulopathy (blood not able to clot leading to bleeding)

What is the overall outcome of Biliary Atresia?

Ninety percent of children can survive into adulthood with the help of medical support, Kasai procedure and subsequent liver transplant. 20-40% can survive into adulthood without liver transplant. The outcome mainly depends upon the timing of Kasai procedure, the centre at which it is done and the skills of the pediatric surgeon. Hence early diagnosis is a key factor. Biliary Atresia is a surgical emergency in a newborn. If you find a baby with jaundice beyond 2 weeks of life, advice the parents to get the baby evaluated or to visit our site for medical advice.

Published articles from India

1. http://www.indianpediatrics.net/oct2001/oct-1144-1148.htm

K.L. Narasimhan, S.K. Chowdhry, Kim Vaiphei, R. Samujh, J.K. Mahajan, B.R.Thapa, K.L.N. Rao. Outcome of Biliary Atresia: results from a prospective study. Indian Pediatrics 2001; 38: 1144-1148. 

2. http://www.indianpediatrics.net/sep1996/729.pdf

Yachha SK, Khanduri A, Kumar M, Sikora SS, Saxena R, Gupta RK, Kishore J. Neonatal Cholestasis syndrome : an appraisal at a tertiary centre. Indian Pediatr 1996; 33: 729-734.

3. http://www.ncbi.nlm.nih.gov/pubmed/?term=Agarwala+S%2C+Mitra+DK.+Biliary+atresia

Agarwala S, Mitra DK. Biliary Atresia- the current management. Indian J Pediatr 1996; 63: 719-724.

4. http://www.ncbi.nlm.nih.gov/pubmed/11519282

Gupta DK, Srinivas M, Bajpai M. AIIMS clinical score: a reliable aid to

Distinguish neonatal hepatitis from extra hepatic Biliary Atresia. Indian J

Pediatr. 2001 Jul;68(7):605-8.

5. http://www.jiaps.com/article.asp?issn=0971-9261;year=1999;volume=4;issue=2;spage=84;epage=89;aulast=Gupta;type=0

Gupta DK, Dave S, Lall A, Srinivas M, Aggarwal M, Bajpai M, et al. Experience with Biliary Atresia disease at AIIMS during the past decade. J Indian Assoc Pediatr Surg 1999; 4: 84-89

6. http://www.indianpediatrics.net/august2000/august-845-851.htm http://www.ncbi.nlm.nih.gov/pubmed/10951633

Indian Academy of Pediatrics. Pediatric Gastroenterology Subspecialty Chapter. Consensus report on neonatal cholestasis syndrome. Pediatric Gastroenterology Subspecialty Chapter of Indian Academy of Pediatrics. Indian Pediatr. 2000 Aug; 37(8):845-51.