Billiary Atresia

Question 1

Obliterative cholangiopathy includes …..

Answer 1

Cystic and noncystic.

The cystic disorders include the different types of choledochal cysts

The noncystic forms are different variants of biliary atresia in addition to neonatal sclerosing cholangitis.

Question 2

What is cystic biliary atresia…..?

Answer 2

Cystic biliary atresia is an uncommon variant of biliary atresia (about 10–20% of cases) and has a relatively favorable prognosis particularly with early surgery

Question 3

Differential features between choledochal cyst and cystic biliary atresia…..?

Answer 3

Cystic biliary atresia disorder is often misdiagnosed as a choledochal cyst.

However, it can be differentiated by the absence of epithelial lining in biliary atresia as well as the lack of communication with the intrahepatic bile ducts as seen on intraoperative cholangiography.

Question 4

What are the are 3 major variants of noncystic biliary atresia…..?

Answer 4

Biliary atresia classified according to the area of involvement

Type I: Atresia of the distal bile duct with patent proximal extrahepatic bile duct.

Type IIa: Atresia of the common hepatic duct.
Type IIb: Atresia of the common hepatic duct, cystic duct, and common bile duct.

Type III: Nonpatency of the entire extrahepatic biliary system and intrahepatic bile ducts at the hilum.

Question 5

Biliary atresia can also be classified into 3 categories based on the presence or absence of associated anomalies….what r those. ?

Answer 5

The most common type, known as perinatal biliary atresia(70%)

Biliary atresia splenic malformation (BASM) syndrome(15%)

The third type ..congenital malformations such as choledochal cysts, kidney anomalies, and cardiac defects(15%)

Question 6

Perinatal biliary atresia…….?

Answer 6

Affecting about 70% of the patients

It is not associated with other anomalies or malformations.

The patients may not be jaundiced at birth.

An evolving process leads to progressive jaundice and acholic stools

Question 7

Biliary atresia splenic malformation (BASM) syndrome……?

Answer 7

Seen in about 15% of the cases,

Associated with heterotaxia malformations including situs inversus, malrotation, polysplenia, interrupted inferior vena cava, and congenital heart disease.

Usually carries a poor prognosis.

Question 8

Incidence of biliary atresia……

Answer 8

Biliary atresia has been detected in 1 in 10,000-15,000 live births.

Biliary atresia is more common in East Asian countries

patients may be born term or preterm

Question 9

In patients with biliary atresia, ultrasound can detect…..

Answer 9

Associated anomalies such as abdominal polysplenia and vascular malformations.

The gallbladder either is not visualized or is a microgallbladder in patients with biliary atresia

Question 10

Ultrasonographic triangular cord sign……?

Answer 10

Which represents a cone-shaped fibrotic mass cranial to the bifurcation of the portal vein may be seen in patients with biliary atresia

Question 11

HIDA…….?

Answer 11

Hepatobiliary scintigraphy with technetium-labeled iminodiacetic acid derivatives is a sensitive but not specific test for biliary atresia

Question 12

——— is the most valuable procedure in the evaluation of neonatal hepatobiliary diseases and provides the most reliable discriminatory evidence.

Answer 12

Percutaneous liver biopsy

Question 13

Biopsy features in Biliary atresia……?

Answer 13

Biliary atresia is characterized by bile ductular proliferation, the presence of bile plugs, and portal or perilobular edema and fibrosis, with the basic hepatic lobular architecture intac

Question 14

In neonatal hepatitis biopsy findings…….?

Answer 14

There is severe, diffuse hepatocellular disease, with distortion of lobular architecture, marked infiltration with inflammatory cells, and focal hepatocellular necrosis; the bile ductules show little alteration.

Giant cell transformation is found in infants with either condition

Question 15

Giant cell transformation on biopsy significance….?

Answer 15

Giant cell transformation is found in infants with either condition and has no diagnostic specificity

Question 16

All patients with suspected biliary atresia should undergo…..?

Answer 16

Exploratory laparotomy and direct cholangiography to determine the presence and site of obstruction

Question 17

The procedure of choice in no correctable lesion biliary atresia…?

Answer 17

Hepatoportoenterostomy (Kasai) procedure

Question 18

Success rate of Kauai procedure……?

Answer 18

The success rate for establishing good bile flow after the Kasai operation is much higher (90%) if performed before 8 wk of life

Question 19

Role of steroid after kasai procedure…..?

Answer 19

The use of steroids following the Kasai procedure has not been shown to improve the patient or the native liver survival rates

Question 20

Degenerative neuromuscular syndrome ……..?

Answer 20

A degenerative neuromuscular syndrome is found in patients with chronic cholestasis, caused by vitamin E deficiency; affected children experience progressive areflexia, cerebellar ataxia, ophthalmoplegia, and decreased vibratory sensation

Question 21

T he recommended initial dose Ursodeoxycholic acid therapy…….?

Answer 21

T he recommended initial dose is 15 mg/kg/24 hr.

Inhibition of the apical sodium dependent bile acid transporter prevents the reabsorption of bile acids in the terminal ileum, is currently under investigation, and may prove to be of therapeutic benefit to relieve pruritus and improve the quality of life.

Question 22

For patients with advanced liver disease, hepatic transplantation has a success rate…….?

Answer 22

> 90%

Question 23

………..Correctable metabolic causes of advanced liver disease by liver transplant.

Answer 23

α1antitrypsin deficiency,
tyrosinemia, and
Wilson disease