Biliary Atresia Treatment in Delhi, India

Babies with biliary atresia usually appear to be healthy when they are born. Initial symptoms of this condition typically occur within the first two weeks to two months of life. Symptoms comprise of:

• Light beige coloured stools (Normal stool colour for infants is yellow, green, or brown).

• Jaundice

• Dark brown urine

• Swelling around the belly area

• Difficulty putting on weight

• Ascites, i.e., fluid accumulation in the belly

• Liver failure after several months if this condition goes untreated.

The exact causes of biliary atresia are not fully known, but some of them comprise:

• Inflammation (swelling) and scarring due to issues with the immune system

• Infection due to any virus

• Exposure to harmful chemicals

• Mutations or Genetic changes (Biliary atresia is not inherited from the baby's parents).

To diagnose biliary atresia, the doctor goes through the baby's medical history and conducts a physical examination. A paediatric hepatologist, a medical professional who specializes in treating kids with liver diseases, may also recommend specific tests, which are as follows:

• Blood tests to assess the bilirubin levels and ascertain any possible signs of liver damage

• Ultrasound is a diagnostic procedure that emits high-frequency sound waves through the tissues of the body. The echoes are recorded and altered into images of various body organs.

• The liver biopsy includes the extraction of cells from the liver for examination.

• Hepatobiliary scan, a radioactive substance or tracer is injected into the blood circulation. The baby may have biliary atresia if the tracer is not evacuated through the liver.

• Cholangiogram is a procedure where a dye is injected into the gallbladder in order to examine whether it passes through the bile ducts. Further, an X-ray is performed to observe whether the dye normally flows into the intestine and the liver. In infants having biliary atresia, the dye does not usually evacuate out of the gall bladder due to the blocked ducts.

Biliary atresia cannot be managed with medicines. A Kasai procedure, also called a hepatoportoenterostomy, is performed. The Kasai procedure is an operation to restore the flow of bile from the liver into the intestine. It is termed after the surgeon who developed and started this.

The doctor removes the damaged ducts outside of the liver, which are called extrahepatic ducts, and recognizes other smaller ducts that are still open and functioning well and draining bile. The doctor then joins a coil of the intestine to this portion of the liver to restore the flow of bile directly from the remaining bile ducts into the intestine.

After this procedure, infants are kept in the hospital for seven to 10 days for observation and healing. Long-term antibiotic therapy is advised to decrease the risk of infection, and additional medications may be added to promote bile flow and maximize the success of the operation.

The Kasai procedure is not a complete cure for kids with biliary atresia, but it does permit babies to grow and have fairly good health for several to sometimes many years.

Success with the Kasai procedure is based on the following:

• Age. The younger infants have a higher success rate for surgery compared to others who are 3 to 4 months old. In those cases, surgery is unlikely to be of much help.

• Amount of cirrhosis, scarring, and damage of liver tissues post-surgery.

• The number and size of microscopic ducts present in the scarred tissue that can function to drain bile.

• The nutritional aspect of the baby at the time of transplant (sufficient vitamins, high-calorie diet)

Liver Transplant

If there is still inadequate bile flow achieved with the Kasai procedure, liver transplantation is ultimately the only option left. A liver transplant operation involves the removal of the damaged liver and replacing it with a new liver from a donor.

Risk factors associated with surgery performed for this condition include:

• Bleeding

• Cholangitis, i.e., infection associated with the bile ducts and liver

• Opening or cavity in the intestine can cause a leakage of the intestinal contents into the belly

• Blockage of the intestine caused by scarring
  
  

Complications post-surgery are relatively low. Most problems that occur are due to the progression of liver disease. Some complications are as follows:

• Infection after the completion of the Kasai procedure

• Itching

• Bruising of skin

• Fluid retention

• Nasal bleeding

• Fluid retention

• Enlargement of veins in the stomach or oesophagus

If the baby does not undergo surgery, it will usually not survive after two years without getting a liver transplant. After the Kasai procedure, the survival rate of a baby without a liver transplant depends mainly on when the procedure was carried out and the status of the liver at the time of surgical intervention. Even with successful surgery, many patients require a liver transplant at some point in their lives.

Reviewed by Dr. Vikram Kumar, Principal Consultant - Paediatric Liver Transplantation, Gastroenterology & Hepatology on 02-Dec-2022.

• Liver Cirrhosis

• All you need to know about liver transplantation