Choledochal cyst - Clinicals, Diagnosis, and Management

Hepato-Pancreatico-Biliary

Clinicals - History

Fact Explanation
Age of presentation This is common problem in infancy and childhood, but about 20% of cases are seen in adults. This is a rare disease, more common in Asia- Japan, with a female predominance. Usually diagnosis is made during childhood. Though the etiology is not very clear, pancreatobiliary abnormalities are common associations . Age of presentation
This is common problem in infancy and childhood, but about 20% of cases are seen in adults. This is a rare disease, more common in Asia- Japan, with a female predominance. Usually diagnosis is made during childhood. Though the etiology is not very clear, pancreatobiliary abnormalities are common associations .
Patients can be asymptomatic for years. They can be asymptomatic for life, but majority of patients will experience symptoms at some point in time. Patients can be asymptomatic for years.
They can be asymptomatic for life, but majority of patients will experience symptoms at some point in time.
Abdominal pain Cystic dilatation and stricture formation causes stasis of bile. This results in stone formation and biliary infections leading to ascending cholangitis. This obstruction causes abdominal pain. Type III choledocal cysts cause gastric outlet obstruction by blocking the lumen or
intussusception . Pain is in right hypochondrium, its radiation is to epigastrium, right iliac fossa and back. Pain is vague, but can be coliky in nature when the cause is an obstruction by stone or stenosis.
Abdominal pain
Cystic dilatation and stricture formation causes stasis of bile. This results in stone formation and biliary infections leading to ascending cholangitis. This obstruction causes abdominal pain. Type III choledocal cysts cause gastric outlet obstruction by blocking the lumen or
intussusception . Pain is in right hypochondrium, its radiation is to epigastrium, right iliac fossa and back. Pain is vague, but can be coliky in nature when the cause is an obstruction by stone or stenosis.
Fever Ascending cholangitis results in fever. This is a common complication of choledochal cysts specially with pancreatitis. Fever
Ascending cholangitis results in fever. This is a common complication of choledochal cysts specially with pancreatitis.
Yellowish discoloration of skin, sclera and the mucous membranes Obstructive jaundice results in this discoloration , this is intermittent, with long standing disease and cholangitis this can be of hepatocellular type . There will be other features of obstructive jaundice as pale stools, dark urine, pruritus . Yellowish discoloration of skin, sclera and the mucous membranes
Obstructive jaundice results in this discoloration , this is intermittent, with long standing disease and cholangitis this can be of hepatocellular type . There will be other features of obstructive jaundice as pale stools, dark urine, pruritus .
Non specific, vague symptoms such as intermittent abdominal pains, nausea Non specific vague symptoms are commonly present in adult patients.Complications of Choledocal cysts are pancreatitis, cholangitis, secondary biliary cirrhosis, spontaneous rupture and cholangiocarcinoma. Cholangiocarcinoma is well known to occur in choledocal cysts, this incident increases with age. So early diagnosis and treatment is a good prognostic factor . Non specific, vague symptoms such as intermittent abdominal pains, nausea
Non specific vague symptoms are commonly present in adult patients.Complications of Choledocal cysts are pancreatitis, cholangitis, secondary biliary cirrhosis, spontaneous rupture and cholangiocarcinoma. Cholangiocarcinoma is well known to occur in choledocal cysts, this incident increases with age. So early diagnosis and treatment is a good prognostic factor .
Vomiting In type III cysts this is due to obstruction. In other cases this is due to the pressure it causes on the anterior abdominal wall . Vomiting
In type III cysts this is due to obstruction. In other cases this is due to the pressure it causes on the anterior abdominal wall .
Upper GI bleeding Choledochal cysts with intrahepatic involvement, they can have secondary biliary cirrhosis. In this situation patients can present with signs of portal hypertension. Splenomegaly, upper GI bleeding & manifestations of pancytopenia are found . Upper GI bleeding
Choledochal cysts with intrahepatic involvement, they can have secondary biliary cirrhosis. In this situation patients can present with signs of portal hypertension. Splenomegaly, upper GI bleeding & manifestations of pancytopenia are found .

Clinicals - Examination

Fact Explanation
Yellowish discoloration of skin, sclera and mucous membranes Cystic dilatation and stricture formation causes stasis of bile.Obstructive jaundice results in this discoloration. This is intermittent, with long standing disease and cholangitis this can be of hepatocellular type. neonates usually present with obstructive jaundice and abdominal masses. Adult presentation is pain, fever, nausea, vomiting and jaundice. Pruritus causing scratch marks will also found as there is jaundice. Yellowish discoloration of skin, sclera and mucous membranes
Cystic dilatation and stricture formation causes stasis of bile.Obstructive jaundice results in this discoloration. This is intermittent, with long standing disease and cholangitis this can be of hepatocellular type. neonates usually present with obstructive jaundice and abdominal masses. Adult presentation is pain, fever, nausea, vomiting and jaundice. Pruritus causing scratch marks will also found as there is jaundice.
Febrile Ascending cholangitis results in fever. Febrile
Ascending cholangitis results in fever.
Palpable abdominal mass On palpation there will be a right hypochondric mass which is cystic, smooth, mobile & discrete. pain, jaundice and abdominal mass is the classical triad of findings in choledochal cysts.. Palpable abdominal mass
On palpation there will be a right hypochondric mass which is cystic, smooth, mobile & discrete. pain, jaundice and abdominal mass is the classical triad of findings in choledochal cysts..
Splenomegaly Choledochal cysts with intrahepatic involvement, they can have secondary biliary cirrhosis. In this situation patients can present with signs of portal hypertension. Splenomegaly, upper GI bleeding and menifestations of pancytopenia are found. Splenomegaly
Choledochal cysts with intrahepatic involvement, they can have secondary biliary cirrhosis. In this situation patients can present with signs of portal hypertension. Splenomegaly, upper GI bleeding and menifestations of pancytopenia are found.
Ascitis This is secondary to portal hypertension and malignancy . Ascitis
This is secondary to portal hypertension and malignancy .

Investigations - Diagnosis

Fact Explanation
Ultra sound scan abdomen This is the initial step towards confirming. Sensitivity 71%- 97% . It shows a cystic mass in right upper quadrant of abdomen. This is not very effective in type III and V cysts though. Advantages are it is non invasive, non expensive method. But it has limitations such as bowel gas, overlying structures. Cyst size also underestimated due to probe pressure . This is a very useful investigation method in prenatal diagnosis also. Ultra sound scan abdomen
This is the initial step towards confirming. Sensitivity 71%- 97% . It shows a cystic mass in right upper quadrant of abdomen. This is not very effective in type III and V cysts though. Advantages are it is non invasive, non expensive method. But it has limitations such as bowel gas, overlying structures. Cyst size also underestimated due to probe pressure . This is a very useful investigation method in prenatal diagnosis also.
CT scan abdomen More accurate. This helps in planning surgical management. identify cyst wall thickness caused by malignant changes. These scans have a risk of nephrotoxicity and hepatotoxicity because of contrast & radiation
exposure. Has 93% sensitivity to visualize biliary tree.
CT scan abdomen
More accurate. This helps in planning surgical management. identify cyst wall thickness caused by malignant changes. These scans have a risk of nephrotoxicity and hepatotoxicity because of contrast & radiation
exposure. Has 93% sensitivity to visualize biliary tree.
ERCP - endoscopic retrograde cholangiopancreatography This helps in accurate diagnosis. Employ in classifying the cyst, measuring the cyst & locating the anatomical relationships prior to the surgery. This is an invasive procedure, can cause cholangitis and
pancreatitis. There are 6 types of Choledochal cysts. Classification by Alonso- Lej (1954), modified by Todani (1977). I-Most common type- 80%- 90%, segmental dilatation of common bile duct is seen. II-cystic duct shows diverticulum. III-choledochocele- this is the least common type -2%. IV-the second common group. IVa-Multiple dilatations of intra and extrahepatic biliary tree is seen. IVb -only extra hepatic duct dilatations. V- Caroli's disease -cystic involvement of intrahepatic
biliary tree.
ERCP - endoscopic retrograde cholangiopancreatography
This helps in accurate diagnosis. Employ in classifying the cyst, measuring the cyst & locating the anatomical relationships prior to the surgery. This is an invasive procedure, can cause cholangitis and
pancreatitis. There are 6 types of Choledochal cysts. Classification by Alonso- Lej (1954), modified by Todani (1977). I-Most common type- 80%- 90%, segmental dilatation of common bile duct is seen. II-cystic duct shows diverticulum. III-choledochocele- this is the least common type -2%. IV-the second common group. IVa-Multiple dilatations of intra and extrahepatic biliary tree is seen. IVb -only extra hepatic duct dilatations. V- Caroli's disease -cystic involvement of intrahepatic
biliary tree.
MRCP- magnetic resonance cholangiopancreatography "Gold standard" for diagnosis.This replace ERCP specially in children. This is a non invasive method of investigation with less
complications.
MRCP- magnetic resonance cholangiopancreatography
"Gold standard" for diagnosis.This replace ERCP specially in children. This is a non invasive method of investigation with less
complications.
technetium -99 HIDA scan Commonly used scan. View the continuity with the bile ducts. Sensitivity of this test depends on type of cyst. Type I- 100% , type IVa 67%. In neonates this differentiate biliary atresia from choledochal cysts technetium -99 HIDA scan
Commonly used scan. View the continuity with the bile ducts. Sensitivity of this test depends on type of cyst. Type I- 100% , type IVa 67%. In neonates this differentiate biliary atresia from choledochal cysts
Mild abnormalities in liver functions These tests are serum bilirubin, alkaline phosphatase, gamma- glutamyltransferase, alanine & aspartate aminotransferases. These will show changes but none are specific.With obstructive jaundice Bile salts & pigments will be found in urine in absence of urobilinogen. High direct bilirubin levels is seen in serum. Mild rise in SGPT is also found. Serum alkaline phosphatase is always elevated in obstructive jaundice( >30 K.A. units) . Mild abnormalities in liver functions
These tests are serum bilirubin, alkaline phosphatase, gamma- glutamyltransferase, alanine & aspartate aminotransferases. These will show changes but none are specific.With obstructive jaundice Bile salts & pigments will be found in urine in absence of urobilinogen. High direct bilirubin levels is seen in serum. Mild rise in SGPT is also found. Serum alkaline phosphatase is always elevated in obstructive jaundice( >30 K.A. units) .

Investigations - Management

Fact Explanation
Bio chemical tests- Liver function tests These tests are done to early detection of potential cholangiocarcinoma. Patients need close regular follow up for life. With obstructive jaundice bile salts and bile pigments will be found in urine in absence of urobilinogen. High direct bilirubin levels is seen in serum. Mild rise in SGPT is also found. Serum alkaline phosphatase is always elevated in obstructive jaundice( >30 K.A. units) . Bio chemical tests- Liver function tests
These tests are done to early detection of potential cholangiocarcinoma. Patients need close regular follow up for life. With obstructive jaundice bile salts and bile pigments will be found in urine in absence of urobilinogen. High direct bilirubin levels is seen in serum. Mild rise in SGPT is also found. Serum alkaline phosphatase is always elevated in obstructive jaundice( >30 K.A. units) .
Abdominal ultra sound scan These tests are done to early detection of potential cholangiocarcinoma. Patients need close regular follow up for life. Chose for the diagnosis. More than 90% cases its diagnostic & helps in staging also. Tumor is seen as a mass lesion.Bile duct dilatation proximal to the mass lesion is sometimes seen. Abdominal ultra sound scan
These tests are done to early detection of potential cholangiocarcinoma. Patients need close regular follow up for life. Chose for the diagnosis. More than 90% cases its diagnostic & helps in staging also. Tumor is seen as a mass lesion.Bile duct dilatation proximal to the mass lesion is sometimes seen.
CA 19-9 Unresected choledochal cyst increases the risk of cholangiocarcinoma. Advanced disease can be detected with increased levels of CA 19-9. CA 19-9
Unresected choledochal cyst increases the risk of cholangiocarcinoma. Advanced disease can be detected with increased levels of CA 19-9.
Carcinoembryonic antigen (CEA) This shows elevated levels in patients with cholangiocarcinoma. But its low sensitivity and specificity makes it not diagnostic. Carcinoembryonic antigen (CEA)
This shows elevated levels in patients with cholangiocarcinoma. But its low sensitivity and specificity makes it not diagnostic.
Serum amylase Raised in pancreatitis . Pancreatitis is a common presenting feature specially in adults. Its because bile reflux activates pancreatic enzymes . Altered coagulation profile & kidney function. This suggest the severity of presentation . Serum amylase
Raised in pancreatitis . Pancreatitis is a common presenting feature specially in adults. Its because bile reflux activates pancreatic enzymes . Altered coagulation profile & kidney function. This suggest the severity of presentation .
CA 19-9 This is a tumor marker. raised levels give rise to suspicion of malignancy . Choledochal cyst is a risk factor for cholangiocaicinoma. Incidence is higher with Type I and IV cysts. Life time risk is 6%- 30%. CA 19-9
This is a tumor marker. raised levels give rise to suspicion of malignancy . Choledochal cyst is a risk factor for cholangiocaicinoma. Incidence is higher with Type I and IV cysts. Life time risk is 6%- 30%.

Management - Supportive

Fact Explanation
Patient education Choledochal cysts has malignancy potential. Patients need follow up regularly for life. Possible complications, management options & prognosis should be discussed in detail.So educate patient & family about these. Patient education
Choledochal cysts has malignancy potential. Patients need follow up regularly for life. Possible complications, management options & prognosis should be discussed in detail.So educate patient & family about these.

Management - Specific

Fact Explanation
Resection surgery Treatment method depends on the type of the cyst.In type I choledochal cyst this method is recommended. This handles the clinical problem and prevent occurring of Cholangiocarcinoma. The reflux of pancreatic enzymes causes chronic inflammation & then malignant changes of the cystic mucosa, survival rate is minute & patient die with in two years in Cholangiocarcinoma.Widely used surgical technique is cystectomy & reconstruction using Roux-en-Y hepaticojejunostomy . Early complications- anastomotic leak, pancreatic leak due to pancreatic duct injury, bowel obstruction due to intussusception & bowel kinking caused by handling or adhesion formation. Late complications- peptic ulcer disease, cholangitis, biliary calculi, pancreatitis, liver failure & cancer. In older age surgical complications are commonly seen. Resection surgery
Treatment method depends on the type of the cyst.In type I choledochal cyst this method is recommended. This handles the clinical problem and prevent occurring of Cholangiocarcinoma. The reflux of pancreatic enzymes causes chronic inflammation & then malignant changes of the cystic mucosa, survival rate is minute & patient die with in two years in Cholangiocarcinoma.Widely used surgical technique is cystectomy & reconstruction using Roux-en-Y hepaticojejunostomy . Early complications- anastomotic leak, pancreatic leak due to pancreatic duct injury, bowel obstruction due to intussusception & bowel kinking caused by handling or adhesion formation. Late complications- peptic ulcer disease, cholangitis, biliary calculi, pancreatitis, liver failure & cancer. In older age surgical complications are commonly seen.
Endoscopic sphincterotomy Type III cysts have much lower malignant potential. This enables the adequate drainage of both biliary & pancreatic ducts. This helps in achieving a long lasting remission of symptoms. Endoscopic sphincterotomy
Type III cysts have much lower malignant potential. This enables the adequate drainage of both biliary & pancreatic ducts. This helps in achieving a long lasting remission of symptoms.
Ursodeoxycholic acid, bile salt binders In Caroli's disease there is diffuse cystic involvement, so initially medical treatment is considered. Ursodeoxycholic acid, bile salt binders
In Caroli's disease there is diffuse cystic involvement, so initially medical treatment is considered.
Liver transplantation If secondary biliary cirrhosis occurs this is necessary to be done.This is considered in caroli's & type IVa with diffuse intrahepatic involvement. Liver transplantation
If secondary biliary cirrhosis occurs this is necessary to be done.This is considered in caroli's & type IVa with diffuse intrahepatic involvement.
Hepatic resection Localized intrahepatic type IVa and Caroli's disease are considered for this. Hepatic resection
Localized intrahepatic type IVa and Caroli's disease are considered for this.

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