Biliary Atresia

Question 1

What is biliary atresia?

Answer 1

A progressive disorder in which both intrahepatic, and some or all of the extrahepatic, ducts are obliterated (close off)

Question 2

What proportion of neonatal cholestasis cases are the result of biliary atresia?

Answer 2

About ¼

Question 3

Is surgery to relieve outlet obstruction a definitive cure for the problems of biliary atresia?

Answer 3

Generally, no –

It progresses, and liver transplant is often required

Question 4

What surgical procedure is commonly performed for infants with biliary atresia when the extrahepatic biliary tree is missing?

Answer 4

The Kasai

hepatoportoenterostomy

Question 5

Why are biliary atresia patients sometimes so itchy?

Answer 5

High-serum bile acids

Question 6

What is the most important determinant of long-term outcome for biliary atresia patients?

Answer 6

Age at surgery!

The younger, the better – even if the liver disease still progresses

Question 7

What infection is especially likely in biliary atresia patients?

Answer 7

Ascending cholangitis

Question 8

What growth & nutrition issues are biliary atresia patients likely to have?
2

Answer 8

1.  Fat-soluble vitamin deficiencies

2.  Poor growth/failure to thrive (FTT)

Question 9

What circumstances indicate that liver transplantation should be considered for a biliary atresia patient?

(4)

Answer 9

1.  Liver failure (of course)
2.  Intractable itching (aka pruritus)
3.  FTT
4.  Significant hemorrhage due to portal hypertension

Question 10

If a biliary atresia patient develops raised yellow “spots” on the skin, what are the spots, and what caused them?

Answer 10

•  Xanthomas (fat deposits in the skin)

*  Hyperlipid states often develop with liver dysfunction

Question 11

If a patient has pain typical of biliary problems, but no biliary abnormalities, what is the likely (biliary) cause?

Answer 11

“Biliary dyskinesia”

Question 12

What are the two other common names for the pain syndrome of “biliary dyskinesia?”

Answer 12

1.  Post-cholecystectomy syndrome (if the patient already had surgery)
2.  Sphincter of Oddi dysfunction

Question 13

Which gender is more likely to suffer from biliary dyskinesia?

Answer 13

Females

as usual, with biliary issues

Question 14

How can biliary dyskinesia be managed medically (at least temporarily)?

Answer 14

Smooth muscle relaxants (nitro-based meds & calcium channel blockers)

Question 15

What medications should be avoided in a biliary dyskinesia patient?

Answer 15

Narcotics (especially codeine)

they could worsen sphincter of Oddi spasms