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Biliary stasis -> Stone formation->Biliary stasis -> Stone formation->Infection(Cholangitis).Infection(Cholangitis).

Associated with a) Congenital hepatic fibrosisAssociated with a) Congenital hepatic fibrosisb)Poly cystic liver c)Rarely cholangiob)Poly cystic liver c)Rarely cholangiocarcinoma.carcinoma.

TREATMENT:TREATMENT:1.Antibiotics to counter cholangitis.1.Antibiotics to counter cholangitis.2.Removal of stones.2.Removal of stones.

3.If the disease is confined to one lobe of liver,3.If the disease is confined to one lobe of liver,Lobectomy is indicated.Lobectomy is indicated.

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CHOLEDOCHAL CYSTS.CHOLEDOCHAL CYSTS.

Aneurismal dilatation of bile ducts.Aneurismal dilatation of bile ducts.Rare, 1 in 1,50,000.Rare, 1 in 1,50,000.

M:F = 1:4.M:F = 1:4.Majority of cases presents in infancyMajority of cases presents in infancyand few in adulthood.and few in adulthood.

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Aetiology.Aetiology.

Exact aetiology is not known.Exact aetiology is not known.Proposed are:Proposed are:1.Partial biliary obstruction and weakness of 1.Partial biliary obstruction and weakness of

ductal wall.ductal wall.2.Abnormal recanalisation.2.Abnormal recanalisation.3.Trauma.3.Trauma.4.Stones-> recurrent cholangitis ->post4.Stones-> recurrent cholangitis ->post

inflammatory fibrosis and stenosis.inflammatory fibrosis and stenosis.5.A.P.B.C.(Abnormal pancreatico-biliary5.A.P.B.C.(Abnormal pancreatico-biliaryconfluence)confluence)

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TODANI- ALANSO-LEJTODANI- ALANSO-LEJCLASSIFICATION.CLASSIFICATION.

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TODANI-contd.TODANI-contd.

Todani Modification of Alonso-Lej Classification of Todani Modification of Alonso-Lej Classification of Choledochal CystsCholedochal Cysts Type I:Type I: Dilation of the extrahepaticDilation of the extrahepaticbiliary tree. Type I dilations are further classified accordingbiliary tree. Type I dilations are further classified accordingto the shape of the affected segment into type Ia: cysticto the shape of the affected segment into type Ia: cysticdilation; type Ib: focal segmental dilation; type Ic, fusiformdilation; type Ib: focal segmental dilation; type Ic, fusiformdilation.dilation. Type II:Type II: Diverticular dilation of the extrahepaticDiverticular dilation of the extrahepaticbiliary treebiliary tree Type III:Type III: Cystic dilation of the intraduodenalCystic dilation of the intraduodenalportion of the common bile duct (choledochocele)portion of the common bile duct (choledochocele) TypeTypeIVa:IVa: Dilation of the extrahepatic and intrahepatic biliaryDilation of the extrahepatic and intrahepatic biliarytreetree Type IVb:Type IVb: Dilation of multiple sections of theDilation of multiple sections of theextrahepatic bile ductsextrahepatic bile ducts Type V:Type V: Dilation confined to theDilation confined to theintrahepatic bile ducts (Caroli's disease)intrahepatic bile ducts (Caroli's disease)

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Todani-contd.Todani-contd.Type I: 82%.Type I: 82%.

Type II: 3%.Type II: 3%.

Type III: 5%.Type III: 5%.

Type IV: 9%.Type IV: 9%.

Type V: 1%.Type V: 1%.

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Pathology.Pathology.

Cysts may be solitary or multiple.Cysts may be solitary or multiple.

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

Wall is composed of thick fibrous tissue,Wall is composed of thick fibrous tissue,

lined by cuboidal epithelium and is oftenlined by cuboidal epithelium and is oftenulcerated in adults.ulcerated in adults.

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Diagnosis:Diagnosis:

Presents as mass or biliary obstruction.Presents as mass or biliary obstruction.Classical triad of RUQ pain, mass/abdomenClassical triad of RUQ pain, mass/abdomen

with jaundice are found only in 50% of with jaundice are found only in 50% of patients.patients.Most of the adults and children present withMost of the adults and children present with

jaundice and may have vomitings due to jaundice and may have vomitings due toextraneous compression of duodenum byextraneous compression of duodenum bythe cyst.the cyst.

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Investigations:Investigations:

U/S; E.R.C.P & M.R.C.P.U/S; E.R.C.P & M.R.C.P.Angiogram.Angiogram.

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Treatment:Treatment:Only surgical.Only surgical.

Type I: Cyst excision + Roux-en-y choledochoType I: Cyst excision + Roux-en-y choledocho jejunostomy. jejunostomy.

Type II: Cyst excision + closure of defect over Type II: Cyst excision + closure of defect over T-tube.T-tube.

Type III: Small cysts by endoscopicType III: Small cysts by endoscopicsphincterotomy and larger cysts by opensphincterotomy and larger cysts by open

sphincterotomy.sphincterotomy.Type IV: Different techniques depending uponType IV: Different techniques depending uponlocation of cysts.location of cysts.

Type V: On similar lines of Carollis disease.Type V: On similar lines of Carollis disease.

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Complications:Complications:

Spontaneous rupture- especially in children.Spontaneous rupture- especially in children.

Cholangitis.Cholangitis.

Secondary biliary cirrhosis (15%).Secondary biliary cirrhosis (15%).

Cholangio carcinoma (8%).Cholangio carcinoma (8%).