Biliary atresia Passmed notes - breadth

Question 1

What is Biliary Atresia?

Answer 1

A pediatric condition involving obliteration or discontinuity within the extrahepatic biliary system, leading to obstruction of bile flow and neonatal cholestasis.

Cholestasis is defined as stagnation, or at least a marked reduction, in bile secretion and flow.

Question 2

What are contributing factors to Biliary Atresia?

Answer 2

Infectious agents, congenital malformations, and retained toxins within the bile.

Question 3

Is Biliary Atresia more common in females or males?

Answer 3

More common in females.

Question 4

At what age does Biliary Atresia typically present?

Answer 4

Neonatal children; perinatal form in the first two weeks, and postnatal form within the first 2-8 weeks of life.

Question 5

What is the incidence of Biliary Atresia?

Answer 5

Occurs in 1 in every 10,000-15,000 live births.

Question 6

What is Type 1 Biliary Atresia?

Answer 6

The proximal ducts are patent, but the common duct is obliterated.

Question 7

What is Type 2 Biliary Atresia?

Answer 7

Atresia of the cystic duct with cystic structures found in the porta hepatis.

Question 8

What is Type 3 Biliary Atresia?

Answer 8

Atresia of the left and right ducts to the level of the porta hepatis, occurring in >90% of cases.

Question 9

What are common symptoms of Biliary Atresia in neonates?

Answer 9

Jaundice extending beyond two weeks, dark urine, pale stools, appetite and growth disturbance.

Question 10

What examination signs are found in biliary atresia

Answer 10

Signs:
Jaundice
Hepatomegaly with splenomegaly
Abnormal growth
Cardiac murmurs if associated cardiac abnormalities present

Question 11

Which investigations are necessary for biliary atresia

Answer 11

Investigations:
- Serum bilirubin including differentiation into conjugated and total bilirubin
- Liver function tests (LFTs) including serum bile acids and aminotransferases
- Serum alpha 1-antitrypsin
- Sweat chloride test
- Ultrasound of the biliary tree and liver
- Percutaneous liver biopsy with intraoperative cholangioscopy

Question 12

What findings are typical in serum bilirubin tests for Biliary Atresia?

Answer 12

Normal total bilirubin, abnormally high conjugated bilirubin.

Question 13

What would LFTs including serum bile acids and aminotransferases show?

Answer 13

Liver function tests (LFTs) icare usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis

Question 14

Why do we do a serum alpha 1-antitrypsin

Answer 14

Deficiency may be a cause of neonatal cholestasis

Question 15

why do we do a sweat chloride test?

Answer 15

Cystic fibrosis often involves the biliary tract

Question 16

What will an ultrasound of the biliary tree and liver show?

Answer 16

May show distension and tract abnormalities

Question 17

Which investigation is a definitive diagnostic tool for Biliary Atresia?

Answer 17

Percutaneous liver biopsy with intraoperative cholangioscopy.

Question 18

What is the definitive treatment for Biliary Atresia?

Answer 18

Surgical intervention to dissect abnormalities into distinct ducts and create anastomosis.

An anastomosis is a surgical connection between two structures.

Question 19

What medical interventions are used following surgery for Biliary Atresia?

Answer 19

Antibiotic coverage and bile acid enhancers.

Question 20

What are potential complications of Biliary Atresia treatment?

Answer 20

Unsuccessful anastomosis, progressive liver disease, cirrhosis, hepatocellular carcinoma.

Question 21

What is the prognosis for Biliary Atresia with successful surgery?

Answer 21

Good, but liver transplantation may be required in the first two years of life if surgery fails.