CONGENITAL ANAMALIES OF BILIARY SYSTEM.

PRESENTED BY:-ARYAJA .A. S

3RD PROF. BAMS

GALL BLADER1. Absence of gall bladder-This is extremely rare. Intra hepatic gall

bladder & left sided gall bladder must be ruled out before making such diagnosis.

1. cystic ducts may be present.2. There may be bilobed G.B with a single cystic duct. Duplication of the

gall bladder with 2 separate 3. Accessory GB may be situated on the left side& its cystic duct may

empty into the left hepatic duct rather than common duct. Collelithiasis & chollecystitis may involve 1 GB while other GB remains un effected.

4. Phrygian cap of the fundus may be responsible for some pathologies& detected in 5% of cholecystographies .

5. Flottng gall bladder occurs when the organ is almost completely invested by peritonium.

6. Gall bladder may be left sided with cystic duct entering into left hepatic duct or common hepatic duct.-Retrodisplacement.

7. The Gall bladder may be totaly intra hepatic .Partial or completely intra hepatic GB is associated with increased chance of cholelithiasis.

Hepatic ductsAccessory hepatic ducts are present in

approximately 15% of cases .1. Large duct is mainly single & drains a portion of

right lobe of liver. It joins the right hepatic duct, common hepatic duct or infundibulum of gall bladder.

2. Small ducts of cholecystohepatic ducts may drain directly from the liver in to body of gallbladder .

CYSTIC& HEPATIC ARTERY Anomalies of these are not common.

1. A large accessory left hepatic A may originate from the left gastric artery.

1. The right hepatic artery may originate from the superior mesenteric artery .

2. There may be 2 hepatic arteries –one originating from the common hepatic A & other from superior mesenteric A.

3. The cystic artery may originate from left hepatic A or the junction of the left & right hepatic arteries .

4. CysticA passes in front of common hepatic duct, rather than to the right to posterior to this duct.

5. There may be double cystic arteries ,both arising from the right hepatic A or may have abnormal origins.

6. Cystic A arises from the gastro duodenal A.7. The right hepatic A is adherent to the cystic duct & neck of the gall bladder.8. The right hepatic artery may course a tortuous turn in front of the cystic

duct ,so that while clamping the cystic duct the right hepatic A may be clamped causing accidental liver infarction.

9. A caterpillar hump or Moynihan’s hump-The right hepatic artery is sometimes tortuous & cystic artery becomes short .

CYSTIC DUCT1. Cystic duct may be absent and the gall bladder is joined to the common

hepatic duct .2. The hepatic duct may be quite long &its union with the common hepatic duct

may be as low as just as above the duodenum.3. The cystic duct may be even longer & may join the common hepatic duct

behind the duodenum.4. Cystic duct is adherent to the common hepatic duct & actual union is quite low.5. The junction between the cystic duct common hepatic duct may be quite high.6. Cystic duct may drain into right hepatic duct.7. Cystic duct crosses anterior to the common hepatic duct &joins it posteriorly.8. Cystic duct crosses anterior to the common hepatic duct &joins it anteriorly.9. Cystic duct may drain into the left hepatic duct or right hepatic duct.

CHOLEDOCHAL CYST Congenital cystic abnormalities may occur through out

entire billiary system, starting from the intra hepatic biliary radicles to the end of common bile duct.

The extra hepatic cystic dilations involving the biliary systemic congenital anamolies has been classified by Alonzo-Lei et al.

Type A is dilatation of the extrhepatic ducts. Type B is rare disorder in which cystic dilatation takes the

form of a diverticulum & is connected with a small stalk to hepatic duct, gall bladder or common bile duct.

Type C is the choledochocele i.e .cystic dilatation involving only intra duodenal portion of common bile duct& this is the least common variety.

Alonzo-Lei type A

It is the most common variety of congenital cyst of extra hepatic biliary tract.

There are 3 major varieties in this group;1. Cystic dilatation involving the entire common bile duct

& common hepatic duct with cystic duct entering the choledochal cyst.

2. Diffuse dilatation of common bile duct.3. A small cystic dilatation of distal common bile duct.

AETIOLOGY It is still not known ,but there are many

speculations. One such is that the supra duodenal diverticulum results from a localised perforation in the bile duct. Another speculum is that fusiform dilatation results from distal obstruction & destruction of the proximal duct epithelium by pancreatic juice when both bile duct &pancreatic duct open commonly at the ampula of vater. Other speculations are not very significant.

CLINICAL FEATURES This condition occurs 4 times more frequently in

females . The classic trade of this condition is a

combination of upper abdominal pain ,obstructive jaundice &fever.

Special investigations

1. Intravenous cholangiography often out lines the choledochal cyst.

2. Now a days non –invasive techniques like ultrasonography has been more popular in diagnosing this condition.

3. MRCP [Magnetic Resonance Cholangiopancreatography]

4. Transphepatic cholangiography .

COMPLICATIONS If untreated this condition proves fatal. It will leads to complete biliary

obstruction ,ascending cholangitis &secondary biliary cirrhosis.

It may undergone rupture ,which occurs frequently during pregnancy leading to diffuse peritonitis.

TREATMENT Early operation is the treatment of choice. Alonzo –Lej type A –Excision of the cyst coupled

with Roux-en-Y anastomosis of jejunum to proximal normal duct known as hepaticodocho-jejunostomy.

Alonzo-Lej type B&C are treated by excision of the cyst , if required ,following by anastomosis either choledochodudenostomy or choledochojejunostomy

CONGENITAL BILIARY ARTESIA The bile duct first develop as solid

structure .Gradually solid core becomes vacuolated &the vacuoles coalesce to establish the lumen.

Arrest in the solid stage causes biliary Artesia. TYPES Type 1-Common bile duct. Type 2-Commonhepatic duct . Type 3-Artessia of right& left hepatic ducts.

CLINICAL FEATURES. It present as jaundice at birth or in neonatal period. Due to the absence of bile in the gut, me conium is not

bile stained, Hence, stools are pale. Gradually due to the back pressure liver enlarges. Enlarge of spleen. Steatorrhoea,pruritis &clubbing are the other features.

Special investigations Blood examination Urine examination.

TREATMENT Surgical drainage of bile is the only available treatment

provided patient bile duct or radical is seen. Anastomosis of Roux-en-y loop of jejunum to dilated bile duct.

Some times excision of bile duct tissue up to the liver capsule should be done followed by Roux-en y anastomosis. This is called Kasai’s Porto enterostomy.

Liver transplantation is the choice when there is Artesia of the intra hepatic duct.

COMPLICATIONS Reurrent cholangitis giving rise to hepatic fibrosis

giving rise to hepatic fibrosis. Billiarycirrhosis & portal hyper tenssion.

CAROLI’S DISEASE. It is a congenital dilatation of intra hepatic ducts. In this condition there are multiple , secular

dilatations of the intra hepatic ducts due to intra hepatic Artesia of the biliary ducts.

This condition is present in child hood &in early adult life.

It is non familial.

complications Cholangitis-It occurs due to constant obstruction. Stones-obstruction stasis precipitate stone

formation. Biliary cirrhosis. Cholangio carcinoma.

SPECIAL INVESTIGATIONS

1. Straight X-ray of abdomen- Performed in billiary tract disease. This diagnosis radio-opaque stones in 15% of

cases. This also shows the rare cases of calcification

of the gallbladder &limy bile. Air or gas in the bile duct system can also be

visualized.

2-oral cholecystography This is to visuallise gallbladder by using contrast

medium. Non visualization of gallbladder usually suggest that

gallbladder is diseased & it has lost of concentrating ability of the dye.

Visualization of gallbladder indicates a normal gallbladder.

It still remains the stained procedure for establishing the diagnosis of chronic cholecystitis &cholelithiasis in non-jaundiced patients.

3-Intravenous cholangiogrphy. This technique applied when the patients are

unable to absorb the orally administered contrast medium.

It is probably suitable for visualization of the bile ducts particularly after cholecystectomy.

4-operative cholangiography In this method contrast dye is directly instilled in to the

cystic duct during operation to demonstrate the entire billiary tract.

This technique should be performed in all cases of cholecystectomy in order to 1- know the congenital abnormalities of cystic duct, hepatic duct & common bile duct.(2)to detect stone in the biliary tract particularly.(3)-to know the whether exploration of bile duct is necessary or not.

5-POSTOPERATIVE CHOLANGIOGRAPHY This is done prior to removal of the tube to

demonstrate(1)presence or absence of retained stones (2)patency of common bile duct (3)whether there is free passage to bile in to the duodenum or not.

6-Ultrasonogrphy Gallbladder ultrasonagraphy is now very well

established as the primary method for gallstone detection.

7-Computed Tomography(CTscan) It is useful for patients in whom ultrasonography

is difficult. e.g-obese patients & those who with excessive

bowel gas.

8-Radioisotopic scaning(Cholescintigraphy) A few isotopes have been used which are secreted in the

bile & can define gallbladder function & its pathology . Normally the scan outlines the liver & extra hepatic

billiary tract including the gallbladder & shows the nuclide following into the upper small intestine as well.

This is the diagnostic choice in acute cholecystitis .

9-Radiomanometry Bile duct pressure can be measured &this is

abnormally raised in a few pathological conditions.

10-Choledocoscopy A choledochoscope inserted into the supra

duodenal part of common bile duct may be employed to determine presence or absence of calculi there.

It can be used for removal of stones & bile duct tumor & for biopsy.

11-Precutaneos transhepatic cholangiography (PTC) It shows intra or extra hepatic obstruction due to

various causes . Complications--Haemorrhage, biliaey leakage &

sepsis .

12-Endoscopic Retrograde Cholangiopancreatography. Its main indications are jaundice, billiary tract

problems, pancreatic disease . The main complications are infection

&pancreatitis.