Dr. Muayad Abbas
Lec. 5
Congenital Abnormalities
of the Gall bladder &
Bile Ducts
Thurs. 12 / 11 / 2015
DONE BY : Ali Kareem
مكتب اشور لالستنساخ
2015 – 2016
Congenital Abnormalities of GB Dr. Muayad Abbas
12-11-2015
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Congenital Abnormalities of the Gall bladder & Bile Ducts
o Absence of the gall bladder
o The Phrygian cap
o Floating gall bladder
o Double gall bladder
o Absence of the cystic duct
o usually a pathological, mirizzi syndrom, anomaly and indicates the recent
passage of a stone or the presence of a stone at the lower end of the cystic
duct, which is ulcerating into the common bile duct.
o The main danger at surgery is damage to the bile duct, and particular care
to identify the correct anatomy is essential before division of any duct.
o Low insertion of the cystic duct
o An accessory cholecystohepatic duct
o Ducts passing directly into the gall bladder from the liver(duct of Luschka)
Choledochal cyst
o congenital dilatations of the intra- and/or extrahepatic biliary system.
o Patients may present at any age with jaundice, fever, abdominal pain and a
right upper quadrant mass on examination.
o increased risk of developing cholangiocarcinoma with the risk varying
directly with the age at diagnosis
Classification
Type I cysts are the most
common and account for approximately 75% of patients
Congenital Abnormalities of GB Dr. Muayad Abbas
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o Ultrasonography will confirm the presence of an abnormal cyst
o magnetic resonance imaging (MRI/MRCP) will reveal the anatomy, in
particular the relationship between the lower end of the bile duct and the
pancreatic duct.
o CT is also useful for delineating the extent of the intra- or extrahepatic
dilatation.
o Radical excision of the cyst is the treatment of choice with reconstruction of
the biliary tract using a Roux-en-Y loop of jejunum.
o Complete resection of the cyst is important because of the association with
the development of cholangiocarcinoma.
TRAUMA of biliary tree
o blunt or penetrating abdominal trauma.
o Operative trauma is perhaps more frequent than external trauma.
o physical signs are those of an acute abdomen.
o Management depends on the location and extent of the biliary and
associated injury.
o In the stable patient, a transected bile duct is best repaired by a Roux-en-Y
choledochojejunostomy.
o Injuries to the gall bladder can be dealt with by cholecystectomy.
Congenital Abnormalities of GB Dr. Muayad Abbas
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THE CHOLECYSTOSES (CHOLESTEROSIS, POLYPOSIS,
ADENOMYOMATOSIS AND CHOLECYSTITIS GLANDULARIS
PROLIFERANS)
o This is a not uncommon group of conditions affecting the gall bladder, in
which there are chronic inflammatory changes with hyperplasia of all tissue
elements.
o Cholesterosis (‘strawberry gall bladder’)
o In the fresh state, the interior of the gall bladder looks something like a
strawberry;
o the yellow specks (submucous aggregations of
o cholesterol crystals and cholesterol esters) correspond to the Seeds .
o It may be associated with cholesterol stones.
The interior of a strawberry gall bladder (cholesterosis)
o Cholesterol polyposis of the gall bladder
o These are either cholesterol polyposis or adenomatous change.
o With improving ultrasonography, they are seen more frequently,
o surgery is advised only if they change in size or are longer than 1 cm.
Cholecystitis glandularis proliferans (polyp, adenomyomatosis and intramural
diverticulosis)
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o A polyp of the mucous membrane is fleshy and granulomatous.
o All layers of the gall bladder wall may be thickened, but sometimes an
incomplete septum forms that separates the hyperplastic from the normal.
o
Intraparietal ‘mixed’ calculi may be present.
o These can be complicated by an intramural, and later extramural, abscess.
o If symptomatic, the patient is treated by cholecystectomy
o Diverticulosis of the gall bladder is usually manifest as black pigment stones
impacted in the outpouchings of the lacunae of Luschka.
o Diverticulosis of the gall bladder may be demonstrated by cholecystography,
especially when the gall bladder contracts after a fatty meal.
o There are small dots of contrast medium just
o within and outside the gall bladder . A septum mayalso be present [to be
distinguished from the Phrygian cap
o The treatment is cholecystectomy.
Cholecystogram showing diverticulosis with
dots of contrast medium in the gall bladder
wall.
Typhoid gall bladder
o Salmonella typhi and occasionally, Salmonella typhimurium can infect the
gall bladder.
o Acute cholecystitis can occur.
o More frequently, chronic cholecystitis occurs, and the patient, being a
typhoid carrier, excretes the bacteria in the bile.
o Gallstones may be present (surgeons
Congenital Abnormalities of GB Dr. Muayad Abbas
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o should not give patients their stones after their operation if there is any
suspicion of typhoid!).
o It is debatable whether the stones are secondary to the Salmonella
cholecystitis or whether pre-existing stones predispose the gall bladder to
chronic infection.
o Salmonellae can, however, frequently be cultured from these stones.
o Ampicillin and cholecystectomy are indicated.
PARASITIC INFESTATION OF THE BILIARY TRACT
o Biliary ascariasis
o The round worm, Ascaris lumbricoides, commonly infests the intestine of
inhabitants of Asia, Africa and Central America.
o It may enter the biliary tree through the ampulla of Vater and cause biliary
pain.
o Complications include strictures, suppurative cholangitis, liver abscesses
and empyema of the gall bladder.
o In the uncomplicated case, anti-spasmodics can be given to relax the
sphincter of Oddi, and the worm will return to the small intestine to be dealt
with by antihelminthic drugs.
o Operation may be necessary to remove the worm or deal with complications.
Worms can be extracted via the ampulla of Vater by ERCP.
Clonorchiasis (Asiatic cholangiohepatis)
o The disease is endemic in the Far East. The fluke, up to 25 mm long and 5
mm wide, inhabits the bile ducts, including the intrahepatic ducts.
o Fibrous thickening of the duct walls occurs
o Complications include biliary pain, stones, cholangitis, cirrhosis and bile
duct carcinoma.
o Choledochotomy and T-tube drainage and, in some cases,
choledochoduodenostomy are required.
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o Because a process of recurrent stone formation is set up, a
choledochojejunostomy with Roux
o loop affixed to the abdominal parietes is performed in some centres to allow
easy subsequent access to the duct system.
o Hydatid disease
o A large hydatid cyst may obstruct the hepatic ducts.
o Sometimes,a cyst will rupture into the biliary tree and its contents cause
obstructive jaundice or cholangitis, requiring appropriate surgery
Primary sclerosing cholangitis
o Primary sclerosing cholangitis is an idiopathic fibrosing inflammatory
condition of the biliary tree that affects both intrahepatic and extrahepatic
ducts.
o It is of unknown origin, but association with hypergammaglobulinaemia and
elevated markers such as smooth muscle antibodies and anti-nuclear factor
suggests an immunological basis.
o The majority of patients are between 30 and 60 years of age.
o There appears to be a male predominance and a strong association with
inflammatory bowel disease, especially ulcerative colitis.
Common symptoms
o right upper quadrant discomfort,
o jaundice,
o pruritus,
o fever,
o fatigue and weight loss.
Investigation
Congenital Abnormalities of GB Dr. Muayad Abbas
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o cholestatic pattern in liver function tests
o elevation of serum alkaline phosphatase and gamma-glutamyl transferase
o and smaller rises in the aminotransferases.
o Bilirubin values can be variable and may fluctuate.
Imaging studies such as MRCP or
ERCP may demonstrate
stricturing and beading of the bile
ducts
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o A liver biopsy is helpful in confirming the diagnosis and may help to guide
therapy by excluding cirrhosis.
o The important differential diagnoses are secondary sclerosing cholangitis
and cholangiocarcinoma.
o Medical management with antibiotics,
o vitamin K,
o cholestyramine,
o steroids
o and immunosuppressants such as azathioprine is generally unsuccessful.
o Endoscopic stenting of dominant strictures and, in selected patients with
predominantly extrahepatic disease, operative resection may be worthwhile.
o For patients with cirrhosis, liver transplantation is the best option.
o Five-year survival following transplantation is in excess of 80%.
END …
Done by
Ali Kareem