Biliary Atresia — MCQs

10 questions

In orthotopic liver transplantation, which is the best way to achieve bile drainage in the donor liver

A 5-year-old child is having acute liver failure. Which one of the following criteria is not included in the King's College criteria?

Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

What is the most common cause of conjugated hyperbilirubinemia in infants presenting between 4-8 weeks of age?

Most common indication for liver transplant in children-

Oesophageal atresia may occur as part of the VACTERL group of anomalies. What does "TE" refer to in this context?

Most common indication of liver transplantation in children:-

In a 2 months old infant undergoing surgery for biliary atresia, you would avoid one of the following anaesthetic -

What is the primary condition treated by the Kasai operation?

Q10

An 11-year-old boy was brought to the outpatient clinic with intention tremor and poor scholastic performance. His sister has similar complaints. On examination, hepatomegaly is seen. The eye finding is shown in the image. What is the probable diagnosis?

Biliary Atresia Indian Medical PG Practice Questions and MCQs

Question 1: In orthotopic liver transplantation, which is the best way to achieve bile drainage in the donor liver

A. Donor bile duct with recipient bile duct or Roux-en-Y choledochojejunostomy (Correct Answer)
B. Donor bile duct with jejunum of recipient
C. Donor bile duct with duodenum of recipient
D. External drainage for a few days followed by choledochojejunostomy

Explanation: ***Donor bile duct with recipient bile duct or Roux en Y choledochojejunostomy*** - The most common and preferred method for bile drainage in **orthotopic liver transplantation** is a **duct-to-duct anastomosis** between the donor and recipient bile ducts. - If a primary duct-to-duct anastomosis is not feasible due to size mismatch, damage, or other reasons, a **Roux-en-Y choledochojejunostomy** is performed, which involves connecting the donor bile duct to a Roux limb of the jejunum. *External drainage for few days followed by choledochojejunostomy* - **External bile drainage** is generally avoided in liver transplantation due to increased risks of **infection** and complications like **bile leaks**. - It does not provide a definitive long-term solution for bile flow and necessitates a secondary, more complex surgical procedure for permanent drainage. *Donor bile duct with jejunum of recipient* - Connecting the donor bile duct directly to the jejunum (without a Roux-en-Y limb) would expose the biliary tree to **intestinal contents**, increasing the risk of **ascending cholangitis**. - The Roux-en-Y configuration is crucial to prevent reflux of food and bacteria into the biliary system, which is not achieved by a simple choledochojejunostomy. *Donor bile duct with duodenum of recipient* - Anastomosing the donor bile duct directly to the **duodenum** significantly increases the risk of **reflux of duodenal contents**, including digestive enzymes and bacteria, into the biliary system. - This reflux can lead to severe and recurrent **cholangitis**, stricture formation, and potential graft failure due to chronic inflammation and infection.

Question 2: A 5-year-old child is having acute liver failure. Which one of the following criteria is not included in the King's College criteria?

A. Age < 11 years (Correct Answer)
B. INR > 6.5
C. Jaundice < 7 days before development of encephalopathy
D. Bilirubin > 300 mmol/L

Explanation: ***Age < 11 years*** - Age is **NOT included** in the original King's College criteria for acute liver failure - King's College criteria are based on **biochemical parameters** (INR, bilirubin, pH, creatinine) and **clinical factors** (encephalopathy grade, jaundice-to-encephalopathy interval), not patient age - While **younger age may be a prognostic factor** in pediatric liver failure, it is not part of the formal King's College criteria used to predict poor prognosis or need for transplantation *INR > 6.5* - An **elevated INR > 6.5** (or PT > 100 seconds) is a **key criterion** in King's College criteria for non-paracetamol acute liver failure - Indicates severe **coagulopathy** and hepatic synthetic dysfunction - One of the most important predictors of poor outcome *Jaundice < 7 days before development of encephalopathy* - The **interval from jaundice to encephalopathy** is explicitly included in King's College criteria for non-paracetamol ALF - Jaundice to encephalopathy < 7 days = hyperacute (relatively better prognosis) - Jaundice to encephalopathy > 7 days = subacute (worse prognosis, indicates need for transplant) - This temporal relationship is a **critical prognostic indicator** *Bilirubin > 300 mmol/L* - **Serum bilirubin > 300 μmol/L** (17.5 mg/dL) is explicitly included in King's College criteria for non-paracetamol ALF - Indicates severe **cholestasis** and hepatocellular dysfunction - Part of the multi-parameter assessment for transplant listing

Question 3: Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

A. A-3, B-4, C-2, D-1
B. A-1, B-4, C-3, D-2 (Correct Answer)
C. A-4, B-2, C-3, D-1
D. A-2, B-4, C-3, D-1

Explanation: **A-1, B-4, C-3, D-2** - **Caplan syndrome** was first described in **coal workers** with **rheumatoid arthritis** and progressive massive fibrosis. - **Asbestosis** is often associated with **pleural effusion**, which can be benign or malignant. - **Mesothelioma** typically involves the **lower lobes** of the lungs, specifically the pleura, and is strongly linked to asbestos exposure. - **Sarcoidosis** is characterized by **non-caseating granulomas**, which have a predilection for the **upper lobes** of the lungs. *A-3, B-4, C-2, D-1* - This option incorrectly states that Caplan syndrome involves the lower lobe; **Caplan syndrome** is defined by the presence of large nodules in the lungs of coal workers with rheumatoid arthritis, and their specific lobar distribution is not a defining characteristic. - This option incorrectly states that Mesothelioma has an upper lobe predominance; **Mesothelioma** is a pleural malignancy and typically involves the **lower lobes**, extending along the pleura. *A-4, B-2, C-3, D-1* - This option incorrectly associates Caplan syndrome with pleural effusion; **Caplan syndrome** manifests as rheumatoid nodules in the lungs, not primarily pleural effusion. - This option incorrectly states that Asbestosis has an upper lobe predominance; **Asbestosis** predominantly affects the **lower lobes** of the lungs, causing interstitial fibrosis. *A-2, B-4, C-3, D-1* - This option incorrectly states that Caplan syndrome has an upper lobe predominance; the defining feature of **Caplan syndrome** is the combination of rheumatoid arthritis and pneumoconiosis, not specific lobar involvement. - This option correctly identifies pleural effusion with asbestosis and lower lobe involvement with mesothelioma, but **Caplan syndrome** is not characterized by upper lobe predominance.

Question 4: What is the most common cause of conjugated hyperbilirubinemia in infants presenting between 4-8 weeks of age?

A. Choledochal cyst
B. Biliary atresia (Correct Answer)
C. CMV hepatitis
D. Alpha-1 antitrypsin deficiency

Explanation: ***Biliary atresia*** - Biliary atresia is the most common cause of **conjugated hyperbilirubinemia** in infants aged 4-8 weeks, necessitating prompt diagnosis and surgical intervention (Kasai procedure). - It involves progressive **fibrotic obliteration** of the extrahepatic biliary tree, leading to bile retention and liver damage. *Choledochal cyst* - While a cause of conjugated hyperbilirubinemia, choledochal cysts are **less common** than biliary atresia in this age group and often present with a palpable mass or intermittent pain. - They are congenital dilations of the **bile ducts**, which can lead to bile flow obstruction. *CMV hepatitis* - **Cytomegalovirus (CMV) infection** can cause neonatal hepatitis and conjugated hyperbilirubinemia, but it is typically diagnosed earlier or has other systemic manifestations. - It is an **infectious cause** of liver inflammation, but less frequent than biliary atresia as the leading cause in this specific age range. *Alpha-1 antitrypsin deficiency* - This **genetic disorder** can cause chronic liver disease and conjugated hyperbilirubinemia, but it is not the most common cause in the 4-8 week age range. - It often leads to **cirrhosis** and emphysema later in life, and diagnosis is confirmed by specific enzyme testing.

Question 5: Most common indication for liver transplant in children-

A. Biliary atresia (Correct Answer)
B. Cirrhosis
C. Hepatitis
D. Drug reactions

Explanation: ***Biliary atresia*** - **Biliary atresia** is the leading cause of chronic cholestasis and end-stage liver disease in infants, making it the most common indication for **liver transplantation** in children. - Due to progressive scarring and obstruction of the bile ducts, it leads to early onset **cirrhosis** and liver failure if not surgically corrected or transplanted. *Cirrhosis* - While **cirrhosis** is an indication for liver transplant, it is a *consequence* of various liver diseases, and not a primary diagnosis itself. - In children, **biliary atresia** is often the underlying cause of cirrhosis that necessitates transplantation. *Hepatitis* - **Hepatitis**, particularly viral hepatitis, can lead to liver failure, but it is less common as a primary indication for transplant in children compared to **biliary atresia**. - Many forms of hepatitis in children may resolve or be managed without the need for transplantation. *Drug reactions* - Severe **drug-induced liver injury** (DILI) requiring transplantation is rare in children. - It accounts for a very small percentage of pediatric liver transplant indications compared to congenital conditions like **biliary atresia**.

Question 6: Oesophageal atresia may occur as part of the VACTERL group of anomalies. What does "TE" refer to in this context?

A. Tetralogy of Fallot
B. Thoracic empyema
C. Tracheo-oesophageal fistula (Correct Answer)
D. Talipes equinovarus

Explanation: ***Tracheo-oesophageal fistula*** - The acronym VACTERL stands for **V**ertebral defects, **A**nal atresia, **C**ardiac defects, **T**racheo-**E**sophageal fistula, **R**enal anomalies, and **L**imb defects. - Oesophageal atresia often coexists with a **tracheo-oesophageal fistula (TE)**, which explains the "TE" component in the VACTERL association. *Tetralogy of Fallot* - This is a type of **cardiac defect (C)**, which is covered by the "C" in VACTERL, not "TE." - It involves four specific heart abnormalities but doesn't directly relate to the **oesophageal or tracheal components**. *Thoracic empyema* - **Thoracic empyema** describes a collection of pus in the pleural space, usually due to infection, and is not a congenital anomaly. - It is an **acquired condition** and not part of the VACTERL association. *Talipes equinovarus* - This condition, also known as **clubfoot**, is a **limb defect (L)**. - While it is a recognized part of the VACTERL association, it specifically relates to the "L" and not the "TE."

Question 7: Most common indication of liver transplantation in children:-

A. Primary biliary cirrhosis
B. Biliary atresia (Correct Answer)
C. Hemochromatosis
D. Wilson's disease

Explanation: ***Biliary atresia*** - **Biliary atresia** is a progressive inflammatory destruction of the **extrahepatic biliary tree** in infants, leading to **cholestasis**, cirrhosis, and liver failure, necessitating a liver transplant. - It is the most common cause of **cholestatic jaundice** in infancy and the leading indication for **liver transplantation in pediatric patients**. *Primary biliary cirrhosis* - **Primary biliary cirrhosis (PBC)** is an **autoimmune disease** predominantly affecting **middle-aged women**, causing progressive destruction of small bile ducts. - It is rarely seen in children and would not be the most common indication for pediatric liver transplantation. *Hemochromatosis* - **Hemochromatosis** is a genetic disorder causing excessive **iron absorption and deposition** in various organs, including the liver, leading to damage. - While it can necessitate liver transplantation in severe cases, it is much less common than biliary atresia as an indication in children. *Wilson's disease* - **Wilson's disease** is a rare genetic disorder characterized by impaired copper metabolism, leading to copper accumulation in the liver, brain, and other organs. - Though it can cause liver failure and require transplantation, it is not as common as biliary atresia in the pediatric population.

Question 8: In a 2 months old infant undergoing surgery for biliary atresia, you would avoid one of the following anaesthetic -

A. Propofol
B. Halothane (Correct Answer)
C. Thiopentone
D. Sevoflurane

Explanation: ***Halothane*** - **Halothane** is a potent hepatotoxic agent and should be avoided in patients with **biliary atresia** or other liver conditions. - Its metabolism can lead to the formation of **toxic metabolites** that can worsen pre-existing liver dysfunction. *Propofol* - **Propofol** is primarily metabolized by the liver, but its hepatic clearance is generally **high** and less dependent on liver function than some other anesthetics. - It is often used for induction and maintenance of anesthesia in patients with liver disease, with **dose adjustments** as needed. *Thiopentone* - **Thiopentone** (thiopental) is an ultra-short-acting barbiturate primarily eliminated by **hepatic metabolism**. - While it has a good safety profile for liver disease compared to halothane, careful **dose titration** is needed due to potential for prolonged effects with severe hepatic impairment. *Sevoflurane* - **Sevoflurane** is an inhalation anesthetic with minimal hepatic metabolism and a low potential for hepatotoxicity. - It is often considered a **preferred agent** in patients with liver disease due to its favorable metabolic profile.

Question 9: What is the primary condition treated by the Kasai operation?

A. Biliary atresia (Correct Answer)
B. Choledochal cyst
C. Hepatocellular carcinoma
D. Primary biliary cirrhosis

Explanation: ***Biliary atresia*** - The **Kasai operation**, or **hepatoportoenterostomy**, is the primary surgical treatment for **biliary atresia**, a condition where the bile ducts are blocked or absent. - The procedure aims to establish bile flow from the liver to the small intestine to prevent liver damage. *Choledochal cyst* - A **choledochal cyst** is a congenital dilation of the bile ducts and is typically treated by surgical excision of the cyst and a **Roux-en-Y hepaticojejunostomy**. - While it involves the biliary system, it is a distinct condition from biliary atresia and requires a different surgical approach. *Hepatocellular carcinoma* - **Hepatocellular carcinoma** is a primary liver cancer, and its treatment options range from **surgical resection** and **transplantation** to **chemotherapy** and **radiation**, which are distinctly different from the Kasai operation. - The Kasai operation is not used for malignant conditions of the liver or bile ducts. *Primary biliary cirrhosis* - **Primary biliary cirrhosis** is a chronic autoimmune disease affecting the small bile ducts within the liver, causing progressive cholestasis. - Its management is primarily medical, focusing on symptom control and preventing disease progression with drugs like **ursodeoxycholic acid**, and surgery is not a primary treatment.

Question 10: An 11-year-old boy was brought to the outpatient clinic with intention tremor and poor scholastic performance. His sister has similar complaints. On examination, hepatomegaly is seen. The eye finding is shown in the image. What is the probable diagnosis?

A. Glutaric aciduria
B. Wilson's disease (Correct Answer)
C. Hepatitis A
D. Huntington's chorea

Explanation: ***Wilson's disease*** - The combination of **intention tremor**, **poor scholastic performance** (indicating neurological involvement), **hepatomegaly**, and the **eye finding** (Kayser-Fleischer ring seen in the image) points strongly to Wilson's disease. The sister having similar complaints suggests an **autosomal recessive** inheritance pattern, consistent with Wilson's disease. - The image shows **Kayser-Fleischer ring**, a golden-brown ring at the corneal limbus due to **copper deposition in Descemet's membrane**, which is pathognomonic for Wilson's disease with neurological involvement. - Wilson's disease is caused by mutations in the **ATP7B gene**, leading to impaired copper excretion and accumulation in the liver, brain, and cornea. *Glutaric aciduria* - This is a rare **autosomal recessive metabolic disorder** that primarily affects the brain, leading to **dystonia** and **developmental delay**. - While it can cause neurological symptoms, it typically does not present with **hepatomegaly** or **Kayser-Fleischer rings** as prominent features. *Hepatitis A* - **Hepatitis A** is an acute viral infection of the liver, causing symptoms like fever, fatigue, nausea, vomiting, and **jaundice**. - However, it typically does not cause **intention tremor**, **poor scholastic performance**, or have a familial pattern suggesting an inherited neurological disorder. It also does not cause Kayser-Fleischer rings. *Huntington's chorea* - **Huntington's chorea** is an **autosomal dominant neurodegenerative disorder** characterized by **chorea**, psychiatric symptoms, and cognitive decline, typically manifesting in adulthood. - It does not present with **hepatomegaly** or **Kayser-Fleischer rings** at this age and is not associated with the constellation of liver and neurological symptoms described.

Want unlimited practice?

Get full access to all questions, explanations, and performance tracking.

Start For Free