BILIARY OR (CHOLEDOCHAL )CYSTS

BILIARY OR (CHOLEDOCHAL )CYSTS

Definition

Choledochal cysts are congenital abnormalities of the biliary channels which characterized by cystic dilatation of the biliary tree which either affected the extrahepatic or intrahepatic biliary tree or both affection which can be present at any age from antenatal periods to late in adult life

BILIARY OR CHOLEDOCHAL CYST TYPE I

Causes

There is no definite causes but theories suggestive that its due to reflux of pancreatic juice into the biliary tree related to the long anomalous pancreaticobiliary channel also weakness of the bile duct wall and increased pressure secondary to biliary obstruction are required for biliary cyst formation

Types of choledochal cysts

It can be divided into five types according to Todani modification of the Alonso-Lej classification

Type I cysts

Fusiform saccular or cystic dilatation of the extrahepatic biliary tree

BILIARY OR CHOLEDOCHAL CYST TYPE II

It is the most common type up to 50% of all choledochal cysts

Type II cysts

Saccular or diverticulum of an extrahepatic bile duct or extrahepatic biliary diverticulum

Rare comprising less than 5% of choledochal cysts

Type III cysts choledochocele

Bile duct dilatation within the duodenal wall (choledochocele) or dilatation of extrahepatic intradoudenal biliary tree

less than 10 % or choledochal cysts
Types IV cysts A and B

IV A cysts

Both extrahepatic and intrahepatic saccular or cystic dilatation

BILIARY OR CHOLEDOCHAL CYST TYPE III

Second most common type of cyst seen in adults 30-40%

IV B cysts

Multiple extrahepatic bilary cyst

Less than 5%

Type V cysts Caroli,s disease

Intrathepatic biliary cysts

Less than 10% of choledochal cysts this disease can be associated with periportal fibrosis and liver cirrhosis

Incidence

Affect female more than male

BILIARY OR CHOLEDOCHAL CYST TYPE IV A

affect any age from antenatal to late adults life

vary in size from 2 cm in diameter to giant cysts

Symptoms and signs

These depend on the age of the patient

The classic triad of choledochal cysts which are jaundice abdominal pain in the right upper quadrant and abdominal mass can be present up to 20 % of the patients

Antenatal periods

May discovered during routine abdominal ultrasound during pregnancy which appear as abdominal mass

Neontal and infants

BILIARY OR CHOLEDOCHAL CYST TYPE IVB

Neonatal jaundice yellowish discoloration of the skin and sclera of the eye
Palpable abdominal mass in the right upper quadrant and may be enlarged liver

Children,s

Abdominal pain colicky pain
Palpable abdominal mass in the right upper quadrant
Jaundice
Fever nausea and vomiting may present

Adults

Abdominal mass may difficult to become palpable due to increased abdominal wall muscle development may felt in thin patient
Abdominal pain is common colicky pain in the epigastric or right upper quadrant of the abdomen
Jaundice
Fever nausea and vomiting
picture of pancreatitis

BILIARY OR CHOLEDOCHAL CYST TYPE V

picture of cholangitis

Complications of choledochal cysts

Most dangerous one it is changes to malignancy or carcinoma of both gall bladder called gall bladder carcinoma see here and bile ducts called cholangiocarcinoma see here

Other complications such as liver cirrhosis cholangitis acute pancreatits and cholecystitis

Postoperative complications also cholangitis pancreatitis and sepsis anastomosis leakage biliary strictures intrahepatic stones formation

Investigations

Laboratory

Liver function test

serum biliurbin are elevated to some extend

Alkaline phosphatase and gamma glutamy transferase are common elevated due to obstructive effect of these cysts the transaminases alanine ALT and asparate aminotransferase AST are also elevated but to some extend

Serum amalyse and lipase

may be elevated in patients with acute abdominal pain and signs and symptoms of clinical pancreatitis

Complete blood count

may showing raised white blood cells count leukocytosis in case of cholangitis

Urine analysis

Showing the colour of urine may be dark colour like tea due to associated obstructive jaundice also urobilinogin and uribiurbin may be present

Radiological studies

Abdominal ultrasound and CT scanning of the abdomen

Showing the cystic as mass and CT showing and define the anatomy of the hepatobiliary and pancreatic regions

Magnetic resonance cholangiopancreatography MRCP also MRI

very excellent for diagnosis and define the anatomy of the biliary channels

Percutaneous transhepatic cholangiograpgy PTC

Very important especially in case of type IV cyst also used to placement of transhepatic stents

Endoscopic retragrade cholangiopancreatograpgy ERCP

Which delinated the distal part well but may not define the most proximal biliary anatomy

For more details see here

Treatment of choledochal cysts

Because the choledochal cysts are associated with malignant transformation to cholaniocarcinoma which can present in young age in contrast to typical presentation of cholangiocarcinmoa which present in old age and gall bladder cancer so all patients with type I, II or type IV should have the cysts excised and the mucosa of the cysts should also be removed

In the past enteric drainage of the cyst was performed but this drainage do not decrease the possibility of malignancy and also was associated with biliary stasis and recurrent infection and biliary strictures

Operations of choledochal cysts

Type I cysts

Either surgical or laparocopically

Removal of gall bladder by cholecystectomy operation
The most distal cystic dilatation is identified
and then the common bile duct (CBD ) is then transected at the intrapancreatic portion with extreme care not to injury the pancreatic duct
A standard 60-cm-Roux-en-Y loop is used for an end to side hepaticojejunostomy

Type II cysts

Can easy to treated by simple excision of the cyst and the closure the defect in the wall of the common bile duct in transverse fashion not in longitudinal fashion to avoid narrowing of the CBD

Type III cyst choledochoceles

The risk of malignancy in this type rare
Treated by approached from a lateral duodenostomy in the second part of the duodenum with intubation of the pancreatic and bile duct with small silastic tube to avoid their injuries
then the cyst is excised and the mucosa of the CBD and pancreatic duct are sutured to the duodenal mucosa using interrupted sutures

Sphincteroplasty may performed and the duodenostomy is closed transversely

Type IV cysts

The entire portion of the extrahepatic biliary tree involved should be resected if possible individual reconstruction of the left right and any accessory ducts is necessary if possible

then hepaticojejunostomy is constructed as type I

Type V cysts

Also called Caroli,s disease

Common confined to single liver or hepatic lobe usually the left one
Treated by hepatic resection

large cyst treated by unroofed to Roux-en-Y limb

In some cases the intrahepatic disease result in extensive fibrosis and need for liver transplantation

These patient need for life long follow up for fear of malignancy

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