Biliary Atresia in Children is one of the rare and serious liver conditions. Approximately 2500 to 5000 children are born with this condition in India, and neonatal cholestasis is one of the major causes of this issue, with a survival rate of below 50%.
But why is the mortality rate this high? Even with good treatment like surgery, it can be treated. Because of a lack of knowledge and awareness, most of the parents don’t even know about such a condition.
Let’s understand what biliary atresia is, why it happens in children, and what parents need to know.
What is Biliary Atresia?
It is one of the rarest conditions of the liver and bile ducts, where bile flow from the liver to the intestine is blocked, causing bile to be trapped in the liver.
Cells in the liver help to produce a liquid called bile, which helps to digest fat as well as carry the waste products to the intestine to excrete the body’s toxic substances. But due to the blockage in the intestine, the liver started to be damaged and scarred (cirrhosis), which eventually led to liver failure.
Common Symptoms
The signs indicating the issue started to appear within the first few months after the birth of the baby. Parents in Jaipur need to understand that signals indicating the health concern of their child should not be ignored.
- Chronic Jaundice- the common sign of biliary atresia is persistent jaundice. Jaundice happens due to the buildup of bilirubin because a newborn’s liver is vulnerable and less efficient in removing bilirubin from the blood. As the bile is unable to be transported from the intestine, it leaks in blood. But if the symptoms persist for 3 to 4 weeks, then this is a major red flag, indicating a serious issue with your child’s health.
- Pale Stool and Dark urine- due to high concentration of bilirubin and absence of enough bile in the intestine, infants passes dark coloured or pale stool and urine.
- Enlarged Abdomen- as the blocked bile ducts come back to the liver, causing severe inflammation, and enlargement of the liver and spleen. Also, the accumulation of waste in the abdomen creates pressure, which looks like a swollen stomach.
- Weight Issues- due to poor absorption of nutrition and digestion of fats, infants face challenges in gaining weight and failure to thrive.
Causes of Biliary Atresia
The causes are not completely understood, but there are some of the risk factors which are linked to the issue, and should not be neglected-
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Abnormal Development (Embryonic or Congenital Form)
Abnormal development during pregnancy causes the absence of the gallbladder in some children. Also, some babies are born with a bile duct issue where it does not form completely during fetal development.
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Immune System Malfunction (Autoimmune Response)
Due to a virus like rotavirus or an in-utero development issue, the baby’s immune system mistakenly attacks bile ducts, leading to inflammation, blockage or scarring.
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Infection
Infection during birth can trigger inflammation in the bile ducts, which can also impact the immune system.
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Environmental Factors
There is still active research on this, but exposure to toxins and chemicals during pregnancy also plays a role.
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Genetic
First of all, this issue can not be inherited by other generations, but genes can increase the vulnerability to the issue.
Diagnosis
If jaundice persists, then the doctor also conducts some test which helps them understand the issue with the liver. Here are some of the common tests that are needed to get a correct diagnosis.
- Blood Test- this is to evaluate the abnormalities in liver function. GCT and liver enzymes like AT and AST are analysed. Also, a specialised MMP-7 protein test is conducted, which helps in distinguishing biliary atresia from other neonatal jaundice causes.
- Liver Biopsy- a small tissue is removed from the liver using a needle, which is further examined under miscroscope. If traces of biliary atresia are detected, then surgery is conducted to confirm the caseand treat alongside.
- Abdominal Ultrasound- through first-line imaging, doctors can detect blocked or absent bile ducts. Moreover, some children are born without a gallbladder, which is also examined through this test.
- Surgery and Special X-ray- this is also known as an operative cholangiogram, which is crucial to confirm the case. Once the issue is confirmed, surgery is performed.
Treatment of BA (Biliary Atresia)
Children born with liver disease or biliary atresia need more calories than any other child, because they are unable to digest essential fats, nutrients, vitamins, and protein, which makes their bodies weaker than those of other children.
The only treatment of this health condition is sureyr called Kasai Procedure or Hepatortoenterostomy. Surgery helps to remove damaged ducts and attach the intestine loop to the portion of the liver that allows bile to flow from healthy remaining bile ducts.
The success rate of this procedure 80%, because in some of the cases liver transplant is needed.
That is why, doctors of Cradle Children Hospital always recommend pregnant women to live a healthy and happy life, away from toxins. Because whatever the eats, think, and live in the environment plays a direct role in fetal health and development. Eating and staying healthy is not just a necessity for the mother, but also for the new life she is carrying.
FAQ’s
Is Biliary atresia also linked with some other abnormalities?
Yes, it is linked with other congenital abnormalities, while 80% are linked with perinatal type, and 20% to minority structural problems like syndromic biliary atresia, sleep malformation, cardiac anomalies, intestinal malrotation, vascular anomalies, kidney issues, and cyst dilation of the bile duct.
What are the risks of BA?
This is a life-threatening condition, and without proper diagnosis and treatment, it can be challenging to save a child’s life.
Who is at risk of BA?
Although the possibility of the condition is rare, girls are more vulnerable than boys, because of hormonal, genetic and immunological factors. Premature babies, babies born through mother with type 2 diabetes, and maternal smoking are at greater risk of issues.
What are the complications of biliary atresia?
Infection after surgery, jaundice, and cirrhosis are some of the complication which child may experience. Apart from that, increased pressure in the veins due to BA can cause sudden bleeding in the stomach and intestine, which can become life-threatening for the child.
Conclusion
As of now, you must have understood the complexity of biliary atresia and how this can become a life-altering situation for your baby. Parents play a crucial role in a child’s healthcare. If you notice any signs of health concern in your child, not just related to biliary atresia, it is crucial to get advice from your doctor. Paediatricians know better than us, and their guidance can help you to take extra care of your baby.
Also, if you are looking for a children’s hospital that is renowned in its field and can offer valuable treatment, then Cradle Children’s Hospital is the one. Because compromise is never the option in a child’s care.
