Choledochal Cyst

Choledochal Cyst

Q. 1

Not true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct.

 B

Type 2 is most common

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Q. 1

Not true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct.

 B

Type 2 is most common

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Ans. B

Explanation:

Type 2 is most common [Ref. Schwartz, 9/e p1440 (8/e p1500); Bailey & Love 25/e p1119 (24/e, p1102; 23/e, p974); Maingot’s 11/e chapter 34 (10/e p1709)1

Type I is the most common.

Choledochal cyst

A choledochal cyst is an isolated or combined congenital dilatation of the extrahepatic or intrahepatic biliary tree. Classification of Choledochal cyst

Type I – a type I cyst is characterized by fusifonn dilatation of the bile duct.

– this is the most common type

Type II – a type II cyst appears as a diverticulum protruding from the wall of the CBD.

Type III – a type III cyst is dilatation of the biliary tract within the duodenum; also k/a choledochocele Type IV

Type IV A – multiple dilatation of the intrahepatic and extrahepatic bile ducts.

Type IV B – multiple dilatation involving only the extrahepatic bile ducts.

Type V – consists of multiple dilatation of the intrahepatic bile ducts; also known as Carolis disease.

– Arro’ttss Point to the choledochal cysts

  • Choledochal cysts are seen to be frequently associated with an anomalous junction of the pancreatic and biliary duct.
  • Clinical Presentation

– more common in females than in males (4:1)

– typically choledochal cysts present in children beyond the toddler age group.

the classical triad presents only in a minority of patients; it consists of

– abdominal pain

– jaundice

– abdominal mass

If the disorder is left undiagnosed, patients may develop cholangitis or pancreatitis. Cholangitis may lead to the development of cirrhosis and portal hypertension.

  • Complications caused by choledochal cyst

recurrent cholangitis

pancreatitis

gall stones

cirrhosis with portal hypertension

portal vein thrombosis with resultant portal hypertension

cyst rupture with biliary peritonitis (very rarely)

– malignancy

  • Diagnosis

– ERCP is the most reliable investigation but is invasive

– MRCP is the best noninvasive investigation

  • Treatment
  • Cholecystectomy + Cyst excision with reconstruction via a biliary-enteric Roux-en-Y anastomosis is the treatment of choice for all choledochal cysts except type III (choledochocele) and intrahepatic cysts of type IV & type V
  • (Caroli’s disease)
  • Treatment of type III (Choledochocele)

transduodenal sphincteroplasty or sphincterotomy – cyst excision

  • Treatment of intrahepatic cysts of type IV & V

if the intrahepatic cysts are localized, hepatic lobe is resected

– if the intrahepatic cysts are diffuse only effective treatment is liver transplantation.

  • Choledochal cyst is a risk factor for the following cancers

biliary tree (most common)               – liver

gall bladder                                        – duodenum pancreas


Q. 2

In choledochal cyst, most common type is:

 A

Type 1

 B

Type 2

 C

Type 4

 D

Type 4A

Q. 2

In choledochal cyst, most common type is:

 A

Type 1

 B

Type 2

 C

Type 4

 D

Type 4A

Ans. A

Explanation:

Type 1 is the most common type and constitute about 50% of the choledochal cysts.

Type 5 is dilation confined to intrahepatic bile ducts (caroli’s disease).

Ref: Sabiston, 18th Edition, Page 1577


Q. 3

Choledochal cyst is a dilation or diverticulum of all or a portion of the common bile duct. NOT true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct.

 B

Type 2 is the most common type

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Q. 3

Choledochal cyst is a dilation or diverticulum of all or a portion of the common bile duct. NOT true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct.

 B

Type 2 is the most common type

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Ans. B

Explanation:

Choledochal cysts are congenital cystic dilatations of the extrahepatic and/or intrahepatic biliary tree.

Type I, fusiform or cystic dilations of the extrahepatic biliary tree, is the most common type, making up >50% of the choledochal cysts. 

More than 90% of patients have an anomalous pancreaticobiliary duct junction, with the pancreatic duct joining the common bile duct >1 cm proximal to the ampulla.

On rare occasions, bile peritonitis secondary to perforation of a cyst is seen. 
 
The treatment is complete excision with Roux-en-Y hepaticojejunostomy.
 
Ref: Oddsdottir M., Pham T.H., Hunter J.G. (2010). Chapter 32. Gallbladder and the Extrahepatic Biliary System. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwartz’s Principles of Surgery, 9e. 

 


Q. 4

In case of choledochal cyst, side-to-side choledochoduodenostomy is NOT recommended because of the risk of:

 A

Malignancy

 B

Pancreatitis

 C

Recurrent Cholangitis

 D

Gall stones

Q. 4

In case of choledochal cyst, side-to-side choledochoduodenostomy is NOT recommended because of the risk of:

 A

Malignancy

 B

Pancreatitis

 C

Recurrent Cholangitis

 D

Gall stones

Ans. C

Explanation:

The treatment for choledochal cyst is complete excision with Roux-en-Y hepaticojejunostomy.

The duodenal end of the bile duct should be oversewn without injury to the anomalous entry of the pancreatic duct, limiting the amount of residual biliary tissue at risk for malignancy.

Side-to-side choledochoduodenostomy is not recommended because it is followed by a high incidence of stricture of the anastomosis and recurrent cholangitis.

Cholecystectomy is always performed.

 
Ref: Albanese C.T., Sylvester K.G. (2010). Chapter 43. Pediatric Surgery. In G.M. Doherty (Ed), CURRENT Diagnosis & Treatment: Surgery, 13e.

Q. 5

Conjugated hyperbilirubinemia in infancy seen in ‑

 A

Choledochal cyst

 B

Extra hepatic biliary atresia

 C

Crigler – Najjar disease

 D

a and b

Q. 5

Conjugated hyperbilirubinemia in infancy seen in ‑

 A

Choledochal cyst

 B

Extra hepatic biliary atresia

 C

Crigler – Najjar disease

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e., Choledochal cyst & `b’ i.e., Extra hepatic biliary atresia


Q. 6

True about choledochal cyst is –

 A

Always extrahepatic

 B

T/t is cystojejunostomy

 C

Excision is ideal t/t

 D

Drainage is t/t of choice

Q. 6

True about choledochal cyst is –

 A

Always extrahepatic

 B

T/t is cystojejunostomy

 C

Excision is ideal t/t

 D

Drainage is t/t of choice

Ans. C

Explanation:

Ans is ‘c’ ie Excision is the ideal t/t 

“The treatment of choledochal cyst is surgical excision followed by biliary-enteric reconstruction. There is no role for internal drainage by cystenterostomy, which leaves the cyst wall intact and leads to the inevitable development of cholangitis. Rarely, choledochal cyst can lead to the development of a biliary tract malignancy. This provides a further rationale for complete cyst excision.” 

More about Choledochal cyst

A choledochal cyst is an isolated or combined congenital dilatation of the extrahepatic or intrahepatic biliary tree.

  • Choledochal cysts are seen to be frequently associated with an anomalous junction of the pancreatic and biliary duct.
  • Clinical Presentation

more common in females than in males (4:1)

typically choledochal cysts present in children beyond the toddler age group. the classical triad presents only in a minority of patients; it consists of abdominal pain jaundice

– abdominal mass

If the disorder is left undiagnosed, patients may develop cholangitis or pancreatitis. Cholangitis may lead to the development of cirrhosis and portal hypertension.

Complications caused by choledochal cyst recurrent cholangitis

– pancreatitis gall stones cirrhosis with portal hypertension portal vein thrombosis with resultant portal hypertension

cyst rupture with biliary peritonitis (very rarely)

– malignancy

  • Diagnosis

– ERCP is the most reliable investigation but is invasive

– MRCP is the best noninvasive investigation

  • Treatment

Cholecystectomy + Cyst excision with reconstruction via a biliary-enteric Roux-en-Y anastomosis is the treatment of choice for all choledochal cysts except type III (choledochocele) and intrahepatic cysts of type IV & type V (Caroli’s disease)

  • Treatment of type III (Choledochocele)

– transduodenal sphincteroplasty or sphincterotomy

– cyst excision

  • Treatment of intrahepatic cysts of type IV & V

–  if the intrahepatic cysts are localized, hepatic lobe is resected

– if the intrahepatic cysts are diffuse only effective treatment is liver transplantation.

  • Choledochal cyst is a risk factor for the following cancers

– biliary tree (most common)                 – liver

gall bladder                                       – duodenum

pancreas



Q. 7

Not true regarding choledochal cyst –

 A

Epigastric mass

 B

Jaundice

 C

Pain in abdomen

 D

Cystojejunostomy is t/t of choice

Q. 7

Not true regarding choledochal cyst –

 A

Epigastric mass

 B

Jaundice

 C

Pain in abdomen

 D

Cystojejunostomy is t/t of choice

Ans. D

Explanation:

Ans is ‘d’ ie Cystojejunostomy is t/t of choice 


Q. 8

In case of choledochal cyst, bile diversion into the small intestine is not done because of the risk of –

 A

Malignancy

 B

Pancreatitis

 C

Recurrent Cholangitis

 D

All

Q. 8

In case of choledochal cyst, bile diversion into the small intestine is not done because of the risk of –

 A

Malignancy

 B

Pancreatitis

 C

Recurrent Cholangitis

 D

All

Ans. C

Explanation:

Ans is ‘c’ i.e. Recurrent Cholangitis 

  • Schwartz writes ‑

“The cyst wall is composed of fibrous tissue and is devoid of mucosal lining. As a result, the treatment of choledochal cyst is surgical excision followed by biliary enteric reconstruction. There is no role for internal drainage by cystenterostomy, which leaves the cyst wall intact and leads to the inevitable development of cholangitis. Rarely, choledachal cyst can lead to the development of biliary tract malignancy. This provides a further rationale for complete cyst excision.”

  • Thus we infer that a bypass procedure will inevitably lead to strictures and recurrent cholangitis. This can be prevented by complete excision of cyst and reconstruction procedure.

Though by complete excision we also prevent malignancy (which may occur in future), but this is an added advantage


Q. 9

Not true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct

 B

Type 2 is most common

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Q. 9

Not true about choledochal cyst is:

 A

Associated with anomalous junction of the pancreatic and biliary duct

 B

Type 2 is most common

 C

Surgical removal is the treatment of choice

 D

If ruptures can cause biliary peritonitis

Ans. B

Explanation:

Ans is b i.e. Type 2 is most common 


Q. 10

Clinical features of choledochal cyst in adult are –

 A

Pain, lump and intermittent jaundice

 B

Pain, fever and intermittent jaundice

 C

Pain, lump and prgressive jaundice

 D

Pain, fever and progressive jaundice

Q. 10

Clinical features of choledochal cyst in adult are –

 A

Pain, lump and intermittent jaundice

 B

Pain, fever and intermittent jaundice

 C

Pain, lump and prgressive jaundice

 D

Pain, fever and progressive jaundice

Ans. C

Explanation:

Ans. is ‘c’ i.e. Pain, lump and progressive jaundice


Q. 11

Most common type of choledochal cyst?

 A

Type 1

 B

Type 2

 C

Type 3

 D

Type 6

Q. 11

Most common type of choledochal cyst?

 A

Type 1

 B

Type 2

 C

Type 3

 D

Type 6

Ans. A

Explanation:

Ans. is ‘a’ i.e., Type I 


Q. 12

Saccular diverticulum of extrahepatic bile duct in choledochal cyst is classified as –

 A

Type I

 B

Type II

 C

Type III

 D

Type IV

Q. 12

Saccular diverticulum of extrahepatic bile duct in choledochal cyst is classified as –

 A

Type I

 B

Type II

 C

Type III

 D

Type IV

Ans. B

Explanation:

Ans. is ‘b’ i.e., Type II 


Q. 13

Which of the following does not predispose to Cholangiocarcinoma

 A

Ulcerative colitis

 B

Clonorchis sinensis

 C

Choledochal cyst

 D

Chronic pancreatitis

Q. 13

Which of the following does not predispose to Cholangiocarcinoma

 A

Ulcerative colitis

 B

Clonorchis sinensis

 C

Choledochal cyst

 D

Chronic pancreatitis

Ans. D

Explanation:

Answer is D (Chronic pancreatitis) :

Chronic pancreatitis does not predispose to cholangiocarcinoma.



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