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Dr. Kant Shah
- Jan. 15, 2025
Biliary atresia is rare, and knowing that your child has a rare condition can be overwhelming for parents. However, with the right treatment and knowledge about the condition, you can ensure your child remains healthy and happy.
What is Biliary Atresia?
Biliary atresia is an innate condition in newborn babies, which is rare but can be a serious liver disease in infants. The liver produces a liquid called bile, which helps digest fat. The bile ducts carry bile from the liver to the small intestine, where digestion and absorption take place. In infants with biliary atresia, due to the atypical development of the bile ducts, they become blocked. As a result, bile cannot flow from the liver to the small intestine and gets trapped in the liver. This leads to scarring and damage to the liver cells (cirrhosis), which eventually results in liver damage. If untreated, it may even lead to liver failure.
How Common is Biliary Atresia?
Biliary atresia occurs in approximately 1 in 10,000 to 15,000 infants and is more common in girls than in boys. The exact cause of the condition is not fully understood, but some potential causes may include inflammation or swelling, and scarring caused by immune system problems (where the immune system attacks the bile ducts by mistake), viral infections during birth, exposure to harmful chemicals, or mutations (genetic changes that are not inherited from the parents).
Signs & Symptoms of Biliary Atresia
The first sign of biliary atresia is jaundice (yellowing of the skin and eyes), which is caused by an excess of bilirubin (a substance produced when red blood cells break down) in the blood. Generally, newborns have some degree of jaundice during the first week of life. However, if jaundice persists after two weeks, it requires immediate medical attention. Other symptoms include, light beige stools (as normal stool color for infants is yellow, green or brown), dark brown urine, swollen belly (as liver & spleen grow), difficulty gaining weight and Ascites (fluid in belly).
Diagnosis of Biliary Atresia
Biliary atresia is diagnosed through multiple tests. The doctor will conduct a physical examination and check for signs of jaundice, a swollen belly, or poor growth. Blood samples will be taken to measure liver enzymes and bilirubin levels. The doctor may also perform an ultrasound to examine the liver and bile ducts, a liver biopsy to assess tissue damage, and a surgery called cholangiogram to directly check the bile ducts.
Treatment Options for Biliary Atresia
Biliary atresia cannot be treated with medication, and surgery is the only option. Two types of surgeries are commonly performed:
Kasai Procedure (Hepatoportoenterostomy)
This is the most common surgery, which creates a new pathway for bile to flow from the liver to the intestine. The goal is to improve bile flow and help the liver function better. The procedure works best if done early, ideally within the first two months of life. However, it does not completely cure the condition, and the child may eventually need a liver transplant.
This surgery can be performed in two ways:
Open Surgery: In this traditional method, the surgeon makes a larger incision in the abdomen to access the liver and bile ducts. The damaged bile ducts are removed, and a loop of intestine is attached between the liver and the small intestine, allowing bile to flow directly. Open surgery is easier to perform and is more effective for complex cases. However, it requires a longer recovery period, more post-operative care, and leads to significant scarring.
Laparoscopic Surgery: In this minimally invasive method, small incisions (2 to 3) are made, and a camera is used to guide the surgeon during the procedure. The steps are similar to those in open surgery, but laparoscopic surgery requires specialized equipment and experience. The benefits include a faster recovery, less post-operative care, and smaller scars.
Liver Transplant
If the Kasai procedure is not effective or if the liver is severely damaged, your child may require a liver transplant. This is a major surgery in which your child receives a new liver from a donor. After the transplant, your child will need lifetime medication to prevent the body from rejecting the new liver.
Post-Surgery Care
After surgery, your child will need constant check-ups to monitor liver function and check for any complications. Babies with biliary atresia often need higher calories and have more metabolic needs than typical infants. They may need special formulas and high-calorie foods and liquids to help with weight gain. If your child undergoes a liver transplant, they will need careful monitoring and medications for the rest of their life to prevent liver rejection.
Biliary atresia is a rare liver disease in newborns where the bile ducts are blocked, preventing bile from flowing from the liver to the small intestine. This blockage can lead to liver damage and scarring.
It occurs in approximately 1 in 10,000 to 15,000 infants and is more common in girls than boys.
The exact cause is not fully understood. Possible factors include:
- Inflammation and scarring of bile ducts.
- Immune system issues attacking the bile ducts.
- Viral infections during birth.
- Genetic mutations or exposure to harmful chemicals.
Common symptoms include:
- Persistent jaundice (yellowing of skin and eyes).
- Light beige stools.
- Dark brown urine.
- Swollen belly.
- Poor weight gain.
- Ascites (fluid buildup in the abdomen).
Diagnosis involves:
- Physical examination for jaundice and swelling.
- Blood tests to check liver enzymes and bilirubin levels.
- Ultrasound to assess the liver and bile ducts.
- Liver biopsy for tissue analysis.
- Cholangiogram surgery to examine bile ducts directly.
No, biliary atresia cannot be treated with medication. Surgery is the only treatment option.
Yes, with early detection, proper treatment, and continuous medical care, most children can lead healthy lives.
Conclusion
Biliary atresia is a rare yet serious condition, but with early detection and timely treatment, your child can grow up healthy. Parents should be vigilant and not ignore any signs of jaundice, poor weight gain, or other symptoms. With the right treatment and care under the guidance of a pediatric surgeon, your child will have a good chance of thriving.
