congenital anomalies of hepato biliary system

1. CONGENITAL ANAMALIES
OF BILIARY SYSTEM.
PRESENTED BY:-ARYAJA .A. S
3RD
PROF. BAMS

10. GALL BLADER
1. 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.

11. Hepatic ducts
Accessory 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 .

12. 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 .

13. CYSTIC DUCT
1. 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.

14. 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.

15. 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.

16. 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.

17. 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.

18. 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 .

19. 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.

20. 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

21. 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.

22. 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.

23. Special investigations
 Blood examination
 Urine examination.

24. 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.

25. COMPLICATIONS
 Reurrent cholangitis giving rise to hepatic fibrosis
giving rise to hepatic fibrosis.
 Billiarycirrhosis & portal hyper tenssion.

26. 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.

27. complications
 Cholangitis-It occurs due to constant obstruction.
 Stones-obstruction stasis precipitate stone
formation.
 Biliary cirrhosis.
 Cholangio carcinoma.

28. 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.

29. 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.

30. 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.

31. 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.

32. 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.

33. 6-Ultrasonogrphy
 Gallbladder ultrasonagraphy is now very well
established as the primary method for gallstone
detection.

34. 7-Computed Tomography(CTscan)
 It is useful for patients in whom ultrasonography
is difficult.
 e.g-obese patients & those who with excessive
bowel gas.

35. 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 .

36. 9-Radiomanometry
 Bile duct pressure can be measured &this is
abnormally raised in a few pathological
conditions.

37. 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.

38. 11-Precutaneos transhepatic
cholangiography (PTC)
 It shows intra or extra hepatic obstruction due to
various causes .
 Complications--Haemorrhage, biliaey leakage &
sepsis .

39. 12-Endoscopic Retrograde
Cholangiopancreatography.
 Its main indications are jaundice, billiary tract
problems, pancreatic disease .
 The main complications are infection
&pancreatitis.