Understanding Choledochal Cysts: A Congenital Anomaly of the Biliary Tract

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LAB
 
CBC : 
WBC :7380  Hb : 12.8   PLT : 252000
AST :279
ALT:210
Total Bili : 6.12
Dirt Bili : 3.91
ALK : 645
ALB : 3
Total Pr : 5
 
 
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Choledochal cyst
 
Choledochal cysts are considered to be congenital
anomalies of the biliary tract characterized by
varying degrees of cystic dilatation at various
segments of the biliary tract (extrahepatic or
intrahepatic).
The frequency of choledochal cysts is about 1 in 15
000 live births  in Western countries and as high as 1
in 1000 live births in Japan.
There is a marked female predominance (4:1)
regardless of the racial origin.
 
 
A choledochal cyst (or congenital bile duct cyst) may
be detected at any age and in any portion of the bile
duct.
 
Choledochal cysts can be classified into five subtypes
.
 
 
Cysts are present in up to 2% of infants with
obstructive jaundice.
 
 
 
Infants present in a manner simulating biliary
atresia and, if unrecognized, may have progressive
disease.
Prolonged obstruction results in biliary cirrhosis,
portal hypertension resulting from cirrhosis, and
pressure on the portal vein by the distended cyst.
Recurrent pancreatitis is an unusual complication of
the malformation.
 
Clinical features
 
The classic triad of intermittent abdominal pain, jaundice,
and right epigastric  mass
varies in incidence; this triad is usually not present in infants
and is uncommon in older children, occurring in about 20%.
Jaundice (conjugated hyperbilirubinemia)  is the common
manifestation.
Abdominal pain may be a presenting symptom, often with
elevated serum amylase levels.
The lesion may be detected at any age, with 18% appearing
before 1 year of age.
Older children may have mild chronic liver disease, which
may reflect variable degrees of common bile duct obstruction.
 
 
In certain patients, the lesion appears to be a true
congenital malformation and is associated with other
anomalies of the biliary tree, such as double common
duct, double gallbladder, and accessory hepatic
ducts, as well as polycystic and hypoplastic kidneys.
Adults with choledochal cyst disease commonly have
acute biliary tract or pancreatic symptoms.
 It is possible that the variability in age and clinical
course represents  two distinct entities: congenital
disease (in infants) versus acquired disease (in older
children)
 
 
 
Spontaneous perforation of a choledochal cyst in
infancy may occur.
The cause of the perforation is postulated to be
biliary epithelial irritation as a result of reflux of
pancreatic juice caused by pancreaticobiliary
malunion associated with mural immaturity, rather
than an abnormal rise in ductal pressure or
congenital mural weakness at a certain point.
 
Classification of bile duct cysts
 
I Cystic dilatation of the common bile duct
Ia Large saccular cystic dilatation
Ib Small localized segmental dilatation
Ic Diffuse (cylindric) fusiform dilatation
II Diverticulum of the common bile duct and/or the
gallbladder
III Choledochocele
IV Multiple cysts
IVa Intrahepatic and extrahepatic (most common form)
IVb Extrahepatic only
V Fusiform intrahepatic dilatations (relation to Carol
disease?)
 
 
Pathogenesis
 
The pathogenesis of choledochal cysts is
undetermined; there are several theories.
Cysts may represent
(1) anomalous union of the common bile duct and the
pancreatic duct proximal  to the sphincter of Oddi, which may
permit reflux of pancreatic enzymes into the common bile duct
with resultant inflammation, localized weakness, and dilatation
(2) congenital segmental weakness of the common bile duct
wall; or
(3) obstruction of the distal common bile duct leading to
dilatation.
 
Diagnosis
 
In most infants, the diagnosis is suggested if non-invasive imaging
studies are undertaken for vague right upper quadrant symptoms.
Ultrasonography should be the initial procedure in the evaluation of
suspected choledochal cyst.
Radiographs of the upper gastrointestinal tract may outline the mass as
it displaces the first and second portion of the duodenum but are
unnecessary.
Ultrasonography may be helpful in the preoperative differential
diagnosis of choledochal cysts in neonates and infants.
Cysts are larger, intrahepatic ducts are dilated, and gallbladders are not
atretic in patients with choledochal cysts compared with patients with
biliary atresia.
 
 
Treatment
 
The goal is complete  surgical excision of the cyst
mucosa, with a Roux-en-Y choledochojejunostomy
proximal to the most distal lesion.
 
Complications and outcome
 
Cholangitis may occur in up to 15% of patients
following surgery, even with the Roux-en-Y
procedure, but is much less common than with direct
anastomosis to the duodenum; the latter procedure
is not advisable.
The high rate of stricture (73%) after cyst
enterostomy is also preventable by total cyst excision
and Roux-en-Y reconstruction.
Pancreatitis is uncommon but may occur secondary
to proximal pancreatic duct or sphincter stenosis or
stones.
 
Malignancy in choledochal cysts
 
Carcinoma has been reported in residual cystic tissue in up to 26%
of patients, an incidence that is 20 times greater than that in the
general population.
The typical malignancy is adenocarcinoma of the bile duct or
gallbladder; less commonly squamous cell carcinoma and
cholangiocarcinoma have been described.
The risk of developing malignancy increases with age, making
complete excision of the cyst and proximal bile duct mucosa an
essential component of the operation in older patients.
Malignant change also may occur in areas of the biliary tree remote
from the cyst.
The increased risk of malignant degeneration and the dismal
prognosis once cancer has developed warrant complete cyst
excision, even in asymptomatic patients, including those with prior
cyst enterostomies.
 
 
 
Thanks for
your attention
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Choledochal cysts are congenital anomalies of the biliary tract characterized by cystic dilatation at various segments. They can lead to complications like biliary cirrhosis and recurrent pancreatitis. Clinical features include jaundice, abdominal pain, and right epigastric mass. Early detection is crucial for appropriate management.


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  1. . . 5 . 5 9 . 4

  2. 2660 5 .

  3. 13 * 22 CBD . .

  4. LAB CBC : WBC :7380 Hb : 12.8 PLT : 252000 AST :279 ALT:210 Total Bili : 6.12 Dirt Bili : 3.91 ALK : 645 ALB : 3 Total Pr : 5

  5. Roux en-Y choleducojejunostomy .

  6. Choledochal cyst Choledochal cysts are considered to be congenital anomalies of the biliary tract characterized by varying degrees of cystic dilatation at various segments of the biliary tract (extrahepatic or intrahepatic). The frequency of choledochal cysts is about 1 in 15 000 live births in Western countries and as high as 1 in 1000 live births in Japan. There is a marked female predominance (4:1) regardless of the racial origin.

  7. A choledochal cyst (or congenital bile duct cyst) may be detected at any age and in any portion of the bile duct. Choledochal cysts can be classified into five subtypes . Cysts are present in up to 2% of infants with obstructive jaundice.

  8. Infants present in a manner simulating biliary atresia and, if unrecognized, may have progressive disease. Prolonged obstruction results in biliary cirrhosis, portal hypertension resulting from cirrhosis, and pressure on the portal vein by the distended cyst. Recurrent pancreatitis is an unusual complication of the malformation.

  9. Clinical features The classic triad of intermittent abdominal pain, jaundice, and right epigastric mass varies in incidence; this triad is usually not present in infants and is uncommon in older children, occurring in about 20%. Jaundice (conjugated hyperbilirubinemia) is the common manifestation. Abdominal pain may be a presenting symptom, often with elevated serum amylase levels. The lesion may be detected at any age, with 18% appearing before 1 year of age. Older children may have mild chronic liver disease, which may reflect variable degrees of common bile duct obstruction.

  10. In certain patients, the lesion appears to be a true congenital malformation and is associated with other anomalies of the biliary tree, such as double common duct, double gallbladder, and accessory hepatic ducts, as well as polycystic and hypoplastic kidneys. Adults with choledochal cyst disease commonly have acute biliary tract or pancreatic symptoms. It is possible that the variability in age and clinical course represents two distinct entities: congenital disease (in infants) versus acquired disease (in older children)

  11. Spontaneous perforation of a choledochal cyst in infancy may occur. The cause of the perforation is postulated to be biliary epithelial irritation as a result of reflux of pancreatic juice caused by pancreaticobiliary malunion associated with mural immaturity, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.

  12. Classification of bile duct cysts I Cystic dilatation of the common bile duct Ia Large saccular cystic dilatation Ib Small localized segmental dilatation Ic Diffuse (cylindric) fusiform dilatation II Diverticulum of the common bile duct and/or the gallbladder III Choledochocele IV Multiple cysts IVa Intrahepatic and extrahepatic (most common form) IVb Extrahepatic only V Fusiform intrahepatic dilatations (relation to Carol disease?)

  13. Pathogenesis The pathogenesis of choledochal cysts is undetermined; there are several theories. Cysts may represent (1) anomalous union of the common bile duct and the pancreatic duct proximal to the sphincter of Oddi, which may permit reflux of pancreatic enzymes into the common bile duct with resultant inflammation, localized weakness, and dilatation (2) congenital segmental weakness of the common bile duct wall; or (3) obstruction of the distal common bile duct leading to dilatation.

  14. Diagnosis In most infants, the diagnosis is suggested if non-invasive imaging studies are undertaken for vague right upper quadrant symptoms. Ultrasonography should be the initial procedure in the evaluation of suspected choledochal cyst. Radiographs of the upper gastrointestinal tract may outline the mass as it displaces the first and second portion of the duodenum but are unnecessary. Ultrasonography may be helpful in the preoperative differential diagnosis of choledochal cysts in neonates and infants. Cysts are larger, intrahepatic ducts are dilated, and gallbladders are not atretic in patients with choledochal cysts compared with patients with biliary atresia.

  15. Treatment The goal is complete surgical excision of the cyst mucosa, with a Roux-en-Y choledochojejunostomy proximal to the most distal lesion.

  16. Complications and outcome Cholangitis may occur in up to 15% of patients following surgery, even with the Roux-en-Y procedure, but is much less common than with direct anastomosis to the duodenum; the latter procedure is not advisable. The high rate of stricture (73%) after cyst enterostomy is also preventable by total cyst excision and Roux-en-Y reconstruction. Pancreatitis is uncommon but may occur secondary to proximal pancreatic duct or sphincter stenosis or stones.

  17. Malignancy in choledochal cysts Carcinoma has been reported in residual cystic tissue in up to 26% of patients, an incidence that is 20 times greater than that in the general population. The typical malignancy is adenocarcinoma of the bile duct or gallbladder; less commonly squamous cell carcinoma and cholangiocarcinoma have been described. The risk of developing malignancy increases with age, making complete excision of the cyst and proximal bile duct mucosa an essential component of the operation in older patients. Malignant change also may occur in areas of the biliary tree remote from the cyst. The increased risk of malignant degeneration and the dismal prognosis once cancer has developed warrant complete cyst excision, even in asymptomatic patients, including those with prior cyst enterostomies.

  18. Thanks for your attention

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