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Congenital Abnormalities

Choledochal Cyst
- It is a Pseudo Cyst.
  1. Choledochal cyst is congenital cystic dilatation of the common bile duct.
  2. Todani classified choledochal cysts into 5 types and several subtypes.
Type I lesions consist of concentric dilatations of the common bile or cystic duct, which may be diffuse or cysti :
  1. Type IA, in which cystic dilatation involves nearly the entire common bile duct (CBD);
  2. Type IB, in which there is more segmental dilatation of the CBD; and
  3. Type IC, in which dilatation is diffuse throughout the CBD and common hepatic duct.
  4. Type I is the most common variant of bile duct cyst.
  5. Type II lesions involve a supraduodenal eccentric dilatation.
  6. Type III cysts, or choledochoceles, are dilatations of the CBD within the muscular portion of the duodenal wall.
  7. Type IVA cysts consist of multiple cystic dilatations that affect both the intra- and extrahepatic ducts; this type accounts for 18% of reported cases.
  8. Type IVB cysts affect the extrahepatic ducts and are much less common.
  9. Type V, or classical Caroli's disease describes cysts of only the intrahepatic ducts. Caroli's   syndrome applies to Caroli's disease in association with congenital hepatic fibrosis.
Extra Edge:     
  1. Bile duct cysts are classified on the basis of site, extent, and shape of the cystic anomaly of the ductal system.
  2. MC choledochal cyst: Type I > Type IV > Type III  (Menonic - 143) 
  1. Caroli's syndrome autosomal recessive trait and associated with adult polycystic renal disease.
  2. Choledochal cysts etiology.
    1. Idiopathic (Most common).          
    2. Anomolous pancreaticobiliary ductal junction (APBDJ), which  is presenting 80% of cases.
    3. Ductal plate anomalies (DPA). This mechanism may involve partial or complete arrest of remodeling of the ductal plate of the larger intrahepatic bile ducts.
  3. Symptoms present mostly in childhood or early adulthood. In infancy, painless or intermittent jaundice with acholic stools is common. Infants may present with nausea, vomiting, and failure to thrive as a result of luminal compression or with a smooth, palpable abdominal mass in the right hypochondrium.
  4. Classical triad consist of pain, jaundice and right upper quadrant abdominal mass.
  5. Most common symptom in adult is abdominal pain.
  6. Complications
    1. Jaundice,                          
    2. Stone formation,                            
    3. Recurrent cholangitis, 
    4. Hepatic abcess,              
    5. Portal vein thrombosis,                                
    6. Pancreatitis
    7. Cyst rupture,                    
    8. Secondary biliary cirrhosis,          
    9. Carcinoma.
    10. Malignant change in 3%-20% of cases.
  7. Diagnosis
    1. Abdominal ultrasound
    2. CT scan are valuable.
    3. ERCP is a definitive method for diagnosing choledochal cyst. 
Extra Edge:
  1. Investigation of choice – ERCP
  2. Best non invasive investigation MRCP.
  • Treatment is usually surgical.
  1. For type I, II & IVb - Complete cyst resection and biliary bypass (Roux-en-Y choledochojejunostomy).
  2. For Type III- ERCP & Sphinctrotomy
  3. For type IVa & VHepatic resection and even liver transplantation
Caroli's Disease (Type V)
  1. Caroli's disease comprises multiple intrahepatic and/or extrahepatic dilatations.
  2. The condition usually presents with either jaundice or cholangitis, and progression to liver failure frequently occurs.
  1. Cholangitis is an acute infection of the biliary tree compounded by presence of bacteria.
  2. CBD stones are the most common cause of obstruction.
  3. Bile stasis may be caused by strictures, stenosis, tumors, or endoscopic manipulation of the CBD.
  4. Partial obstruction is more likely to cause cholangitis than complete obstruction.
    1. Right upper quadrant pain,
    2. Fever Jaundice.
    3. Reynold pentad:-
      1. Mental status changes
      2. Sepsis to the triad
      3. Most common isolated organism E. Coli, klebsiella pneumoniae, streptococcus fecalis and bacteroides fragilis
      4. Transabdominal USG initial investigation of choice.
      5. Sonograms followed by CT scan are used most commonly.
      6. ERCP or percutaneous cholangiogram images and treats the obstruction directly.
      7. Ultrasonography is fairly sensitive for intrahepatic and extrahepatic, including CBD dilation;but it is not reliable for choledocholithiasis.
      8. CT scan: Dilated intrahepatic and extrahepatic ducts and inflammation of the biliary tree are imaged.
    4. Gallstones are visualized poorly with CT scan.
      1. Biliary scintigraphy (HIDA and DISIDA) are functional studies of the gallbladder.
      2. Obstruction of the common bile duct causes nonvisualization of the small intestine. A HIDA scan with complete biliary obstruction does not visualize the biliary tree.
      3. Advantages include its ability to assess function, and positive results may appear before the ducts are sonographically enlarged.
    5. Treatment
  5. Broad-spectrum antibiotics with close observation to determine the need for emergency decompression.
    1. In severely ill patients, treatment is immediate biliary decompression by percutaneous or endoscopic drainage.
    2. Mainstay of therapy is biliary drainage.
    3. Best method to accomplish biliary drainage is ERCP.
Recurrent pyogenic hepatitis (Oriental cholangiohepatitis)
  1. It is characterized by formation of intrahepatic pigmented stones with recurrent exacerbation and remission of abdominal pain, frequently associated with jaundice, chills, and fever.
  2. The cause coliform bacteria or parasites (Clonorchis sinensis), causing pigmented stone formation by inducing the precipitation of bilirubin, acting as nidi for stone formation, or causing biliary strictures that lead to further biliary stasis.
  3. The hallmark of the disease is presence of soft pigmented bilirubinate stones within dilated intra-extrahepatic ducts.
  4. Clinical presentation is characterized by recurrent attacks of right upper quadrant pain, fever, chills, jaundice.
  5. Findings are polymorphonuclear leukocytes, elevated levels of alkaline phosphatase, and excretion of urobilinogen in urine.
  6. USG is highly sensitivity for the detection of cholelithiasis and hepatic ductal dilatation.
  7. CT scan can be helpful when sonographic findings are not definitive or are equivocal.
  8. Direct cholangiography : such as endoscopic retrograde cholangiography, percutaneous transhepatic cholangiography, operative cholangiography, and T-tube cholangiography demonstrates the full spectrum of ductal changes and stones.
  9. Treatment:  
    1. Management of acute cholangitis     
    2. Followed by either drainage and removal of stones using endoscopic, radiologic, or surgical methods  
    3. Hepatic resection for focal disease
Def. Bleeding into the biliary tree.
  1. Pain                                                            
  2. Jaundice                                        
  3. Gastrointestinal haemorrhage
  1. Causes: 
  1. Itrogenic is most common                                        
    1. ERCP / PTC                                                            
    2. Laproscopic / open cholecystectomy.
    3. CBD exploration                                                  
    4. ​Liver biopsy.
  2. Abdominal trauma
  3. Tumor
    1. Hepato cellular cancer                                      
    2. Gall bladder cancer
    3. Cholengiocarcinoma
  4. Inflammatory condition
  5. Vascular malformation
  6. Coagulopathy
  1. Diagnosis:
    1. Retrograde cholangiography may reveal clots in the bile duct,
    2. Hepatic angiography will define the site of haemorrhage. 
    3. Investigation of choice  - CT Angiography
  2. Treatment:
    Selective hepatic angiography with embolization of the bleeding vessel is an effective means of treatment which obviates the necessity for laparotomy.
Bile Duct Strictures
  1. Causes: 
    1. Iatrogenic (M/C causes)  resulting from operative trauma.
    2. Pancreatitis bile duct stones,
    3. Primary sclerosing cholangitis (PSC),
    4. HIV cholangiopathy (Cryptosporidium and cytomegalovirus may be responsible), 
Fig: Strasberg classification of postoperative bile duct strictures
Strasberg Classification of Laparoscopic Biliary Injuries
 Type A · Bile leaks from minor ducts still in continuity with the CBD
  · Includes leakage from cystic duct stump and from a sub-vesical duct of Luschka
  · MC causes of biliary leaks seen after laparoscopic cholecystectomy
 Type B · Occlusion of a part of the biliary tree, almost always an aberrant right sectoral duct
 Type C · Transection without ligation of an aberrant right sectoral duct
 Type D · A lateral injury to an extrahepatic duct
 Type E · Includes biliary strictures, divided into E1 to E5 as classified by Bismuth
The Stewart-Way classification of laparoscopic bile duct injury
Class Criteria
I CBD mistaken for cystic duct but recognized; cholangiogram incision of cystic duct extended into CBD
II Lateral damage to common hepatic duct from cautery or clips placed on duct; associated bleeding, poor visibility
III CBD mistaken for cystic duct, not recognized; CBO, CHO, RHO, LHO transected or resected
IV RHO mistaken for cystic duct, RHA mistaken for cystic artery, RHO and RHA transected; lateral damage to the RHO from cautery or clips placed on ducts
Mirizzi syndrome

  1. Pressure on the common hepatic duct due to a gallstone impacted in the Hartmann pouch or cystic duct results in jaundice and cholangitis.
  2. Choledochal cyst
  3. Recurrent pyogenic cholangitis.
  • Complication:
Bile duct strictures can cause
  1. Ascending cholangitis,                       
  2. Liver abscess,                      
  3. Secondary biliary cirrhosis. 
Extra Edge: Pancreatic cancer is the most common cause of malignant biliary strictures.

History: Most patients with biliary strictures remain asymptomatic until the lumen of the bile duct is sufficiently narrowed. Occasionally, patients may have intermittent episodes of right upper quadrant pain (biliary colic) or features of obstructive jaundice; pruritus, yellow discoloration of skin, and steatorrhea. With chronic cholestasis, xanthomas appear around the eyes, chest, back, and on extensor surfaces.
Patients presenting with cholangitis also may have fever and right upper quadrant tenderness in addition to jaundice (ie, Charcot triad), hypotension, and altered mental status (ie, Reynold pentad).
  • Lab Studies:
  1. Elevated serum alkaline phosphatase
  2. Elevated gamma-glutamyl transpeptidase.
  3. Increases in total and conjugated bilirubin.
Extra Edge: Alkaline phosphatase levels are increased to more than 3-times normal. In malignant strictures causing only partial obstruction (eg, Klatskin tumor), a rise in the alkaline phosphatase level may not be accompanied by a rise in the bilirubin level.
  • Imaging Studies:
  1. The initial study should be an ultrasound.
  2. Magnetic resonance cholangiopancreatography (MRCP)
  3. Abdominal CT scans.
  4. Most sensitive investigation to diagnose biliary leak is HIDA scan.
  5. Gold standard investigation for biliary stricture is PTC.
  6. In some patients, ERCP may be needed for definitive diagnosis and has the advantage of being therapeutic.
  7. HIDA scan is commonly used for the diagnosis of biliary leaks. HIDA scanning can help determine  the clearance of bile across strictures and surgical anastomosis.
  8. HIDA scan findings suggest complete biliary obstruction if the small intestine is not visualized in 60 minutes. HIDA scanning also is useful for distinguishing cholangitis from cholecystitis. 
Bismuth proposed an anatomic classification based on location, into 5 types:
  1. Type 1: Low common hepatic duct stricture. 2 cm of the hepatic duct is intact.
  2. Type 2: Mid common hepatic duct stricture. The hepatic duct stump is < 2 cm.
  3. Type 3: This is a hilar stricture. The common hepatic duct is not involved, but the confluence of right and Left hepatic  ducts is intact.
  4. Type 4: In this type, the hilar confluence is destroyed. The right and left hepatic ducts are separated.
  5. Type 5: The aberrant right sectorial duct is involved, alone or with the CBD.
Extra Edge:
  1. Most common site after open cholecystectomy in the CBD
  2. Most common sit of stricture after lap cholecystectomy in the common hepatic duct, distal to confluence of right and left hepatic duct.
  • Treatment:
    Medical Care: Medical treatment consists of managing complications of bile duct strictures until definitive therapy can be instituted.
  • Surgical Care:
    Endoscopic or percutaneous balloon dilatation and insertion of an endoprosthesis (Sphincterotomy and endoscopic balloon dilation, Endoscopic biliary stenting,  PTC and biliary stenting) or surgery: Operative treatment: Surgical management consists of restoration of biliary enteric continuity, which usually is achieved with a defunctionalized Roux-en-Y jejunal loop by hepaticojejunostomy, choledochojejunostomy or intrahepatic cholangiojejunostomy.
Gallbladder Tumors
  1. More than 80% of patients diagnosed with gallbladder cancer have gallstones.
  2. Adenocarcinoma is the most frequent (80%), and papillary carcinoma has the most favorable prognosis.
  3. More common in female ratio 3:1
  4. Risk factors associated with GB cancer
    1. Gallstones
    2. Anomalous pancreaticobiliary junction
    3. Chronic typhoid infection
    4. Inflammatory bowel disease
    5. Porcelain gall bladder.
    6. Chemicals
  • Methyldopa
  • Oral contraceptives
  • Isoniazid
  1. Pathological features Q
    GB cancer categorised by
    1. Growth patterns into
      • Infiltrative (most common)
      • Nodular
      • Papillary
      • Combined forms
    2. Histologically into
      • Epithelial cell origin (99%)
        • Squamous
        • Adenocarcinoma (most common)
        • Oat cell
      • Mesenchymal origin. Spread by all 4 routes
        • Direct adjacent organ invasion
        • Lymphatic spread
        • Hemotogenous spread
        • Peritoneal drop metastasis
        • Usual mode of spread to the liver is by direct extension and usually involve segment IV and V.
Stage TNM System Modified Nevin system
I Mucosal or muscular invation (T1 No Mo) In situ carcinoma
II Transmural invastion (T2 No Mo) Mucosal or muscular invasion
III Liver invasion <2cm; lymph node metastasis <(T3 N1 Mo) Transmural direct liver invasion
IV (A) Liver invasion <2cm (T4N1 Mo) Lymph Node metastasis
  (B) distant metastasis (TxN2Mo;TXNXM1)  
V - Distant metastasis
  1. Clinical presentation
    1. Pain in the MC-symptom.
    2. Jaundice
    3. Anorexia, weight loss, anemia
    4. Mass in Rt upper quadrant (obstruction of the cystic duct or the neck of gall bladder may lead to hydrops of the gall bladder which present as mass).
  2. Laboratory findings
    1. Increased alkaline phosphatase
    2. Increase bilirubin levels
    3. Elevated CEA and CA19-9 tumor markers
  3. Imaging Studies:
    1. The diagnosis of gall bladder cancer is rarely made preoperatively unless grossly metastatic deposits are seen on preoperative studies.
    2. Computed tomography (CT) is more accurate than ultrasonography (US) for staging purposes. ERCP,
    3. MRCP, and endoscopic ultrasound (EUS) add little to the treatment plan and may not be necessary. Imaging modality of choice CT - Scan
    4. Benign conditions of the gallbladder, such as adenomyomatosis, cholesterol polyps, and hyperplastic polyps, may mimic cancer on pre-operative imaging studies. Polyps larger than 10 mm should be considered to harbor malignant foci until proven otherwise.
    5. Diagnostic Procedures: When indicated in patients with obstructive jaundice, preoperative biliary decompression may be achieved endoscopically or percutaneously, but it should not delay surgery.
    6. Percutaneous biopsy may be undertaken for diagnosis when non-operative and palliative treatment is being planned.
Focal and diffuse thickening of the gall bladder with hyperechoic shadows with comet tail
sign is seen in:  (AIIMS Nov 08)
A. Xantho granulomatous cholecystitis                     
B. Adenomyomatosis

C. Gall bladder polyp                                                    
D. Carcinoma gall bladder

Ans. B



Stage Procedure 5 yr Survival
I Simple Cholecystectomy 85-100%
II Radial cholecystectomy include wedge resection of the GB bed and regional lymphadenectomy of the hepatoduodenal ligament + CBD resection 80-90%
III & IV 1 yr mortality is about 95%

Carcinoma of bile duct
  1. Intrahepatic cholangiocarcinoma
  2. Extrahepatic Cholangiocarcinoma Intrahepatic Cholangiocarcinoma
    1. Arises from biiary channel.
    2. It is a less well differentiated ADENOCARCINOMA typically with an abundant firbous stroma
    1. Bile is NEVER seen in the acini or the cells, whereas mucus formation is common.
    2. It is associated with:
      1. Primary sclerosing cholangitis
      2. Parasite infestation with the liverfiuke, CLONORCHIS SINESIS OR OPISTHORCHES SINESIS.
  • Never resectable.
  1. Biliary diversion can relieve biliary obstruction
  2. Prognosis is poor.
  3. 90% cases are associated with gall bladder stones (usually cholesterol type gall stones)

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