Biliary atresia is a liver disease that only affects infants. In biliary atresia, the tubes (ducts) that carry bile from the liver to the gallbladder are blocked, causing a bile build-up in the liver that damages the organ. It is a rare condition that affects 1 out of 10,000 childbirths and occurs relatively more in girls than boys.
The bile produced and released by the liver helps digest and absorb food. While there is an obstruction in the ducts, causing liver damage, there is a hindrance for the bile to enter the small intestine. So, the required nutrition, such as proteins or vitamins, is not absorbed from our food.
Types of Biliary Atresia
There are two types of biliary atresia.
Perinatal biliary atresia is more common in newborns and occurs 2-4 weeks after birth. Environmental factors may cause this type of biliary atresia.
Fetal biliary atresia is less common in infants. It appears when the baby is still in the mother's womb.
Biliary Atresia Causes
The causes of biliary atresia are not fully understood. As per medical experts, it may occur due to the following:
Exposure to toxic chemicals
Problems with auto-immune mechanisms
Biliary Atresia Symptoms
Babies born with biliary atresia appear normal at birth. However, symptoms develop within two months after childbirth.
Jaundice: Improper liver function causes high bilirubin levels in the bloodstream. This can make the eyes and skin appear yellow.
Dark urine: Excess bilirubin in the blood enters the kidney, which is expelled through urine.
Light-coloured stools: The presence of bile in the intestine gives the stool a brown or green colour. As bile does not enter the intestine because of obstruction, stool often appears grey or white.
Loss of weight
Biliary Atresia Diagnosis and Tests
Doctors perform a physical examination followed by a few diagnostic tests to confirm biliary atresia, as the symptoms are similar to other liver conditions.
Blood Tests for Biliary Atresia
Blood tests are performed to confirm biliary atresia. Doctors look for the following conditions:
High levels of bilirubin
High levels of liver enzymes
Low albumin and total protein
Low blood clotting time
Presence of any viral infection
Blood cultures for bacterial infection
Other Tests for Biliary Atresia
X-ray: A special X-ray, called an operative cholangiogram, checks for an enlarged spleen or liver.
Abdominal ultrasound: A diagnostic imaging test that uses sound waves to make images of the gallbladder, bile ducts, and liver.
HIDA (Hepatobiliary iminodiacetic acid) scan: A radioactive isotope is used to scan the liver and intestine with the help of a nuclear medicine instrument.
Liver biopsy: A biopsy is done by collecting a tissue sample from the child's liver. It helps to rule out whether it is biliary atresia or any other liver problem.
Surgery: In some cases, doctors perform exploratory surgery by making small incisions to examine the bile ducts and liver.
Biliary Atresia Treatment
Biliary atresia can be life-threatening without surgery. Usually, there are two types of surgical procedures to treat this illness. Doctors will take a decision depending on the child’s health.
Kasai procedure: The Kasai procedure is a surgery that connects bile waste from the liver directly to the small intestine by going around the blocked bile ducts. This operation shows successful results if performed before the child is eight weeks old. However, after a few years, the bile is at risk of entering the liver and causing liver damage. In such cases, a liver transplant must be performed.
Liver transplant: In a liver transplant, doctors remove the damaged liver and replace it with a new part or whole liver from a donor. There is a high risk of rejection of the new organs by the body's immune system. So, doctors give antirejection medicines to weaken the immune response. After the transplant, the child has to be under medical supervision until recovery. The nutritional requirements of a child with biliary atresia are higher than an average child. As they have poor digestion because of the unavailability of bile in the intestine, there will be a loss of proteins and vitamins. However, the health condition recovers after the surgery.
Risk Factors of Biliary Atresia
Biliary atresia poses a greater risk for premature infants than full-term babies. African-American and East Asian children are at a greater risk of this rare gastrointestinal disorder.
Complications of Biliary Atresia
Newborns suffering from biliary atresia can suffer from other associated abnormalities of the heart, intestine (malrotation), kidneys (cysts) and enlarged spleen (polysplenia).
Biliary Atresia Prevention
Biliary atresia cannot be prevented since its causes are unknown. Ultrasound scans cannot detect any abnormalities during pregnancy, and this condition can only be seen after birth.