Distinguish Congenital Biliary Atrophy With Physiological Jaundice

Congenital biliary atrophy is one of the causes of jaundice and cholestasis in children. Children at risk of dying before 1 year of age if not detected, treated promptly. This is a congenital disease whose cause is unknown.
The incidence is about 1 in 10,000 newborns. When found, the baby usually operated by Kasai method

. The success of this surgery depends on the age of the child (preferably 2 to 3 months of age), the extent of liver damage and the level and experience of the surgeon.
Congenital biliary atrophy easy to identify?
Congenital biliary atrophy is a rare condition where patients may only have biliary atrophy of the liver but there may be cases of extrahepatic biliary atrophy. The two main manifestations of a child with congenital biliary atrophy are discolored stools and yellowing of the skin and eyes However, the degree of jaundice and the degree of color change of stool depends on biliary atrophy.




Because bile ducts are atrophy, bile pigments and bile salts in the liver cannot follow the bile ducts down the small intestine to digest food normally. That is also the reason why the child's stool is different colors.

Depending on the level of atrophy of the bile ducts, young feces are white like storks (also known as living feces), white as clay or pale yellow. In addition, the child's urine is dark yellow, diapers or clothes can hardly be washed. Another common symptom in children with congenital biliary atrophy is jaundice and yellow eyes. The level of gold increases gradually over time.
If not treated or treated without results, the progression progresses, the child will have signs of cirrhosis such as hepatomegaly, splenomegaly, ascites, collateral circulation, subcutaneous hemorrhage

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What to do to make an accurate diagnosis?
When seeing newborns jaundice lasts long, it is necessary to take the baby to the nearest medical facility for examination and necessary tests to diagnose the cause. Currently, in our country, ultrasound is a valuable method to diagnose congenital biliary atrophy.

If atrophy of the biliary tract atrophy or general hepatic ducts, ultrasound shows that the bile duct is not visible, the bile duct is not visible or dilated, the gallbladder is not seen or shrunk, the size of the gallbladder does not change when fasting, after breastfeeding for 5 minutes and 45 minutes. If atrophy of the end of the common bile duct, ultrasound shows a part of the bile duct outside the liver, you can see the bile ducts in the liver dilate and the gallbladder visible.
In addition, if available at central level health facilities, liver radiography may be performed. This is a test that has a high diagnostic value and requires early diagnosis to make an accurate diagnosis for surgery.
Need to distinguish from any pathology?
Physiological jaundice: Usually exists only in the first 2 weeks after birth, after which the symptoms of jaundice gradually subside to normal.



Anemia caused by congenital or hemolytic hemolytic anemia Characteristics of these diseases are pale, discreet, recurrent anemia, splenomegaly.
In addition, it should be distinguished from the pathologies that cause jaundice in other children such as hepatitis, Gilbert's disease, Crigler's disease - Najjar tsp I and type II, lobar pneumonia, typhoid, regression fever ..., however, all both of these pathologies can be detected by ultrasound when the bile ducts and the gallbladder are still detected.
Kasai method for treatment of congenital biliary atrophy

When congenital biliary atrophy is detected, the baby is usually operated by Kasai method. The Kasai method was proposed by Japanese doctor Morio Kasai (1959). This is surgery to create a bile duct from the liver using part of the small intestine.



The success of this surgery depends on the age of the child (preferably 2-3 months), the extent of liver damage and the level and experience of the surgeon. According to the literature, this measure can reduce the symptoms of the disease, especially jaundice for more than 60% of children.
Age: As an important factor, prognosis at a younger age the better the prognosis. The age of surgery has a high rate of good results is less than 1 month of age or within 2 months of age. Over 2 months of age, the failure rate is very high.
Bile atrophy category: With complete atrophy of the bile ducts, the prognosis is bad, while partial atrophy of the bile ducts is good prognosis.
Cholangitis after surgery: Occurs sooner or later after surgery. Need early detection for treatment and should have additional anti-reflux valve during surgery.




However, some children who have made significant progress after Kasai surgery have relapsed symptoms and suffered severe complications due to obstruction of biliary tract such as hepatomegaly, splenomegaly, varicose veins in visceral, cirrhosis, liver failure, severe infections such as pneumonia, pancreatitis ....

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