CAROLLI’S

DISEASE.(CONGENITAL
DILATATION OF INTRA
HEPATIC BILIARY DUCTS)
1.Rare, congenital, non familial.
2.Charectarised by multiple, irregular, sacular
dilatation of intra hepatic ducts, separated by
segments of normal or stenosed ducts.
 Biliary stasis -> Stone formation->
Infection(Cholangitis).
 Associated with a) Congenital hepatic fibrosis
b)Poly cystic liver c)Rarely cholangio
carcinoma.
TREATMENT:
1.Antibiotics to counter cholangitis.
2.Removal of stones.
3.If the disease is confined to one lobe of liver,
Lobectomy is indicated.
 CHOLEDOCHAL CYSTS.
 Aneurismal dilatation of bile ducts.
 Rare, 1 in 1,50,000.
 M:F = 1:4.
 Majority of cases presents in infancy
and few in adulthood.
 Aetiology.
 Exact aetiology is not known.
Proposed are:
1.Partial biliary obstruction and weakness of
ductal wall.
2.Abnormal recanalisation.
3.Trauma.
4.Stones-> recurrent cholangitis ->post
inflammatory fibrosis and stenosis.
5.A.P.B.C.(Abnormal pancreatico-biliary
confluence)
TODANI- ALANSO-LEJ
CLASSIFICATION.
 TODANI-contd.
 Todani Modification of Alonso-Lej Classification of
Choledochal Cysts Type I: Dilation of the extrahepatic
biliary tree. Type I dilations are further classified according
to the shape of the affected segment into type Ia: cystic
dilation; type Ib: focal segmental dilation; type Ic, fusiform
dilation. Type II: Diverticular dilation of the extrahepatic
biliary tree Type III: Cystic dilation of the intraduodenal
portion of the common bile duct (choledochocele) Type
IVa: Dilation of the extrahepatic and intrahepatic biliary
tree Type IVb: Dilation of multiple sections of the
extrahepatic bile ducts Type V: Dilation confined to the
intrahepatic bile ducts (Caroli's disease)
 Todani-contd.
 Type I: 82%.

 Type II: 3%.

 Type III: 5%.

 Type IV: 9%.

 Type V: 1%.
 Pathology.
 Cysts may be solitary or multiple.

 Size ranges from 2 C.m to giant size.

 Wall is composed of thick fibrous tissue,

lined by cuboidal epithelium and is often
ulcerated in adults.
 Diagnosis:
 Presents as mass or biliary obstruction.
 Classical triad of RUQ pain, mass/abdomen
with jaundice are found only in 50% of
patients.
 Most of the adults and children present with
jaundice and may have vomitings due to
extraneous compression of duodenum by
the cyst.
 Investigations:
 U/S; E.R.C.P & M.R.C.P.
 Angiogram.
 Treatment:
 Only surgical.
Type I: Cyst excision + Roux-en-y choledocho
jejunostomy.
Type II: Cyst excision + closure of defect over
T-tube.
Type III: Small cysts by endoscopic
sphincterotomy and larger cysts by open
sphincterotomy.
Type IV: Different techniques depending upon
location of cysts.
Type V: On similar lines of Carolli’s disease.
 Complications:
 Spontaneous rupture- especially in children.

 Cholangitis.

 Secondary biliary cirrhosis (15%).

 Cholangio carcinoma (8%).