Biliary Atresia

There are many causes of jaundice in children, especially in infants. The topic we will discuss here involves conditions that cause obstruction in the bile ducts and therefore require surgery. Normally, bile is produced in the liver and flows through the bile ducts to the gallbladder and the duodenum (the first part of the small intestine). During this process, excess bile is stored in the gallbladder and released into the duodenum when needed. The substance that gives bile its light color is bilirubin, which is divided into two types: direct and indirect. In obstructive jaundice, direct bilirubin increases. If direct bilirubin exceeds 10% of total bilirubin or is higher than 1.5 mg/dL, it is considered pathological.

Bilirubin is formed by the breakdown of hemoglobin molecules, which are responsible for transporting oxygen in red blood cells. In cells that have completed their life span and are broken down for the production of new cells, hemoglobin goes through various biochemical processes and forms bilirubin.

 

What is Biliary Atresia?

Biliary atresia is a congenital condition in which there is no continuity of the bile ducts. Its incidence is reported as 1 in every 15,000 live births, though it may be more common in certain populations and geographic regions. It is more frequently seen in female infants. The condition is progressive, and diagnosis is generally made around the first month after birth. If diagnosis is delayed, the liver progressively deteriorates, fibrosis develops, and eventually cirrhosis occurs, ending in liver failure.

What Causes Biliary Atresia?

The exact cause is unknown, but several theories suggest potential contributing factors:

• Insufficient recanalization (bile ducts initially form without a lumen in the womb, and failure in the process of forming a lumen may be a factor),
• Ischemic damage (lack of adequate blood supply during bile duct development),
• Toxic damage,
• Infectious causes (e.g., Reovirus Type III),
• Early embryological developmental anomalies.

Is Biliary Atresia Genetic or Familial?

There is no definitive evidence showing it is genetic or familial. However, cases of multiple individuals with biliary atresia within a single family have been reported in the literature. Additionally, some chromosomal anomalies have been identified in affected patients.

Are There Any Associated Anomalies with Biliary Atresia?

Yes, there can be. Commonly associated anomalies include absence of the inferior vena cava, preduodenal portal vein, intestinal malrotation, and polysplenia.

Are There Types of Biliary Atresia?

Yes, there are. It is broadly categorized into intrahepatic and extrahepatic biliary atresia.

How Is Biliary Atresia Diagnosed?

The diagnosis of biliary atresia is made based on medical history and physical examination, and is confirmed with certain tests. Approximately 30% of obstructive jaundice cases in newborns are due to biliary atresia. The most prominent findings include jaundice that first appears in the whites of the eyes and later spreads across the body, turning a greenish-yellow hue in delayed cases; acholic (colorless or chalk-white) stools; and hepatomegaly (enlarged liver). Clinical signs appear within the first week in only about 30% of patients but typically become more evident in the 3rd to 4th week. This delay is due to bile being cleared via maternal circulation in the womb. In history-taking, jaundice lasting more than 1 week in term newborns and more than 2 weeks in preterm newborns should not be considered physiological. Jaundice in the first month may stem from obstructive, infectious, hematologic, or metabolic hepatic parenchymal diseases.

On physical examination, jaundice and acholic stool are observed. The liver initially feels enlarged with sharp edges but becomes smaller and blunter as cirrhosis develops.

Laboratory tests are then conducted, starting with blood work and liver function tests.
Ultrasonography (US) can provide helpful information for diagnosing biliary atresia.
Scintigraphy using Technetium-99m iminodiacetic acid (IDA) analogs (HIDA scan) is very useful in imaging the hepatobiliary system. This test provides information on hepatocyte clearance, hepatobiliary transit, and excretion. Hepatocyte clearance is reduced in hepatocellular diseases. The test’s sensitivity is increased if phenobarbital (5 mg/kg) is administered 5–7 days beforehand. The absence of passage into the intestines suggests biliary atresia.
Percutaneous needle biopsy may also be performed. However, none of these three tests are definitively diagnostic, and some neonatal conditions cannot be clearly differentiated.

Laparoscopic Cholangiography: The patient is prepared for surgery as if a definitive procedure will be performed. In the operating room, laparoscopy is conducted to visually inspect the liver and bile ducts, and imaging is performed to visualize the bile ducts. If atresia is present, definitive surgery is performed; if not, the procedure is terminated. Families must be thoroughly informed before the procedure

(Biliary Atresia) What Conditions Can Biliary Atresia Be Confused With?

It is most commonly confused with neonatal hepatitis and biliary hypoplasia. Additionally, for understanding hepatocellular causes, differential diagnosis should include numerous conditions such as syphilis, toxoplasmosis, listeriosis, hepatitis B, galactosemia, fructosemia, and alpha-1-antitrypsin deficiency, as well as choledochal cyst, biliary sludge, total parenteral nutrition, and masses compressing the bile ducts.

(Biliary Atresia) How Is Biliary Atresia Treated?

Once diagnosed, medical treatment is initiated immediately. The definitive treatment is surgery. Since the disease is progressive, early diagnosis and surgical intervention are critical to success. In biliary atresia cases, due to the rapid development of hepatic fibrosis after 10 weeks, diagnosis should be made, and surgery performed within this time frame. The current surgical procedure for biliary atresia is hepatoportoenterostomy. In late cases or when the surgery is unsuccessful, liver transplantation becomes the only option.

 

Can the KASAI Procedure Be Performed Laparoscopically (Minimally Invasive)?

Yes, it can be performed laparoscopically.

Are There Any Advantages of Laparoscopic Surgery Compared to Open Surgery?

Yes, there are. The main advantages are listed below:

• Less pain
• Less bleeding
• Lower risk of infection
• Smaller or no surgical scars
• Shorter hospital stay
• Faster and easier recovery
• Quicker return to daily activities

How Is the Laparoscopic KASAI Procedure Performed?

Tiny incisions are made in the abdomen to insert carbon dioxide (CO₂) gas, which inflates the abdominal cavity for better visibility. Three small incisions, including one at the belly button, measuring approximately 0.3–1 cm, are made at appropriate sites. Special tubes are inserted through these incisions, and long surgical instruments are passed through the tubes to perform the planned surgery.

However, it is important to note that if performed by inexperienced individuals, laparoscopy can lead to more serious complications.

What Is Done Before the KASAI Procedure?

First and foremost, a thorough medical history is taken and a general physical examination is conducted. Then, the family (mother and father) must be well informed about the preoperative, operative, and postoperative processes. Some tests are performed, which are not standard but are determined based on the patient’s condition. The patient is also evaluated by an anesthesiologist, and both the child and family are informed about the anesthesia process. The patient should not eat or drink anything for 4 hours before surgery.

Are There Any Complications of Hepatoportoenterostomy (KASAI Procedure)?

One of the most significant complications of hepatoportoenterostomy is cholangitis. In this complication, fever, leukocytosis, jaundice, and cessation of established bile flow are observed. If systemic antibiotic and corticosteroid treatment is not effective, surgery is required to reopen the hepatoenteric anastomosis, perform curettage of the hilar region, and recreate the anastomosis. Another complication is portal hypertension due to hepatic fibrosis. In some cases, liver failure may develop to a degree that requires liver transplantation.

Roughly, among patients who undergo the KASAI procedure:

• 1/3 live with minimal issues,
• 1/3 do well until around the age of 10, after which progressive liver damage may necessitate liver transplantation in later years,
• 1/3 do not benefit from the surgery from the beginning and will require liver transplantation.

Are There Factors That Affect the Prognosis of the Disease?

Yes, there are. The most important factor is the age at which the KASAI procedure is performed. Studies have shown that surgeries performed before 60 days of age have better outcomes. Other important prognostic indicators include:

• The patient’s condition after KASAI (e.g., early cholangitis or persistent jaundice indicate poor prognosis),
• The degree of liver damage observed during surgery,
• The level of D-glucuronic acid detected in urine.

How Are Patients with Biliary Atresia Monitored After KASAI Procedure?

These patients should be closely followed by a Pediatric Surgeon and a Pediatric Gastroenterologist. Long-term treatment may require:

• Antibiotics,
• Corticosteroids,
• Vitamin K,
• Ursodeoxycholic acid (Ursafalk),
• And other medications as necessary.

*** The information provided here is organized for the purpose of informing visitors, especially families, through this website. None of the information should be taken as advice or used as a basis for any decisions or actions. Families must have their child examined and evaluated directly by a pediatric surgery specialist