Biliary atresia is a serious condition causing obstruction of bile ducts in infants, leading to cholestasis and potentially liver failure if untreated. The document outlines the etiology, clinical features, diagnostic methods, treatment options, and outcomes of biliary atresia, emphasizing the need for early recognition and surgical intervention to improve prognosis. Surgical options include Kasai's portoenterostomy, and the long-term outlook varies significantly based on age at surgery and post-operative complications.

1. OBSTRUCTIVE JAUNDICE
Dr.B.Selvaraj MS;Mch;FICS;
Professor Of Surgery
Melaka Manipal Medical college
Melaka 75150 Malaysia
BILIARY ATRESIA

2. BILIARY ATRESIA- Objectives
To be aware of possible causes of surgical jaundice in infancy
To be aware of clinical features of biliary atresia
To be familiar with the methods of investigation
To understand the management options
To be aware of the surgical outcome and results

3. BILIARY ATRESIA
Biliary atresia is also known as progressive obliterative
cholangiopathy
Biliary atresia causes a progressive damage of the extrahepatic and
intrahepatic bile ducts with cholestasis because of obstructive
cholangiopathy secondary to inflammation, leading to fibrosis, and if
not recognized and treated, it leads to biliary cirrhosis and liver
failure.
The incidence of biliary atresia is 1:10,000 to 1:15,000 live births
Biliary atresia affects girls more often than boys.

4. BILIARY ATRESIA
Asians and African-Americans are affected more frequently than
Caucasians - more common in Chinese and Japanese.
Biliary atresia should be recognized and distinguished from neonatal
jaundice. Infants with prolonged jaundice should be thoroughly
investigated for biliary atresia.
Biliary atresia should be considered in all neonates with direct
hyperbilirubinemia
A high index of suspicion is important to make the diagnosis of
biliary atresia because surgical treatment by age 2 months has
clearly been shown to improve the outcome and establishment of bile
flow and to prevent the development of biliary cirrhosis.

5. BILIARY ATRESIA-Etiology
The exact cause of biliary atresia is not known and many factors
have been incriminated
1. Reovirus 3 infection.
2. Congenital malformation. Early studies postulated a congenital
malformation of the biliary ducts leading to their obstruction.
3. Congenital cytomegalovirus (CMV) infection.
4. Autoimmunity
5. A possible association with the gene GPC1, which is located on the
longarm of chromosome 2 (2q37).
6. Identification of active and progressive inflammation and
destruction of the biliary system

6. BILIARY ATRESIA-Types
Clinically, biliary atresia occurs in two distinct forms:
 The fetal-embryonic form:
− Appears in the first 2 weeks of life.
− About 10–20 % of affected neonates have associated congenital
defects.
 The postnatal form:
− Appears in neonates and infants aged 2–8 weeks.
− Progressive inflammation and obliteration of the extrahepatic bile
ducts occur after birth.
− Not associated with congenital anomalies.
− Infants may have a short jaundice-free interval.

7. BILIARY ATRESIA-Types
Type I: atresia of the common bile duct
Type IIa: atresia of the common hepatic
duct
Type IIb: atresia of common bile duct,
cystic duct, and common hepatic duct
Type III: atresia of the common bile duct,
cystic duct, and hepatic ducts up to the
porta hepatis. This is the subtype present
in over 90% of patients with biliary
atresia

8. ASSOCIATED ANOMALIES
Associated anomalies are seen in 10 to 20 % of biliary atresia cases.
 Polysplenia syndrome:
 These include:
− Cardiac malformations
− Polysplenia
− Situs inversus
− Absent vena cava
− A preduodenal portal vein
-- Bilobed symmetric liver
-- Bilobed lungs
BASM
Syndrome

9. Clinical Features
Infants with biliary atresia are typically full term
The symptoms are seen usually between 1 and 6 weeks of life
 Conjugated Jaundice which is prolonged
Dark urine because of bilirubin in urine
Clay-colored stools because of absence of stercobilin.- acholic stools
Vitamin K deficiency and coagulopathy
Antenatally detected subhepatic cyst
Cirrhosis (ascites and hepatosplenomegaly), rarely younger than 3
months

10. Biliary Atresia- Investigations
 LFT: Total bilirubin and direct bilirubin are elevated
ALP & GGT are elevated; AST & ALT mildly elevated
Medical causes, such as a1-antitrypsin deficiency, Alagille’s
syndrome, cystic fibrosis and neonatal hepatitis, should be
excluded.
 Biliary USG: whether the gallbladder is atrophic, dilated or
abnormal.
 to exclude other etiologies like choledochal cyst
 Triangular cord sign: Presence of fibrous cone of bile duct
remnant at porta hepatis

11. Triangular Cord Sign
Triangular Cord Sign Micky Mouse Sign

12. Biliary Atresia- Investigations
 Hepatobiliary isotope scan- HIDA scan: In biliary atresia, there is
normal uptake by the liver and failure of secretion of the isotope,
while in neonatal hepatitis there is poor liver uptake of isotope.
Intestinal excretion of the isotope confirms patency of the
extrahepatic bile ducts
Percutaneous liver biopsy: bile ductular proliferation; bile duct plugs;
inflammatory cell infiltrate, hepatocyte giant cell transformation
Intraoperative cholangiography: this procedure definitively
demonstrates anatomy and patency of the extrahepatic bile ducts

13. Investigations- HIDA Scan

14. Investigations- IOC
Intra Operative Cholangiogram

15. Biliary Atresia- Treatment
 Kasai’s portoenterostomy: Once biliary atresia is suspected, surgical
intervention in the form of intraoperative cholangiogram and Kasai
portoenterostomy is indicated.
This procedure is not usually curative, but ideally does buy time until
the child can achieve growth and undergo liver transplantation
A considerable number of these patients, even if Kasai
portoenterostomy has been successful, eventually undergo liver
transplantation

16. Kasai’s Portoenterostomy

17. Kasai’s Portoenterostomy

18. Biliary Atresia- Postop care
 In the immediate postoperative period, Methylprednisolone should
be given for it’s anti-inflammatory and choleretic effects
Ursodeoxycholic acid has also been shown to enhance bile flow.
In order to prevent cholangitis postoperatively, immediate postop
give IV antibiotics, then prophylaxis with trimethoprim–
sulfamethoxazole has been used on a long-term basis.

19. Biliary Atresia- Outcome
 Prognosis is good if operated before 2 months of age
 Risk factors for failure liver fibrosis &Post op cholangitis episodes
1/3rd of pts remain asymptomatic No transplant
1/3 never have bile flow and require early transplant
1/3 initially have good bile flow but subsequently develop cirrhosis
Without surgery or liver transplant life span – 19 months
Death is due to liver failure, bleeding esophageal varices and sepsis

20. Biliary Atresia - Mindmap

21. Differential Diagnosis

22. Infantile Surgical Jaundice- Algorithm

23. THANK YOU
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