Recurrent Pyogenic Cholangitis
Biliary pathogens Clonarchis sinensis, Ascaris lumbroides populate biliary tree – secrete enzyme that hydrolyze water soluble bilirubin glucuronides to form bilirubin, that precipitates to brown pigment stone – obstruct biliary tree to cause recurrent cholangitis and eventually abscess or even cirrhosis
PSC associated with
- Ulcerative Colitis
- Riedel Thyroiditis
PSC cholangiography finding
Chain of lakes, Diverticular like outpouch, multiple short segment strictures
Radiological finding of Benign biliary strictures
Long, Smooth, gradually tapered narrowing
Etiology of Choledochal cysts?
- Presence of an anomalous pancreaticobiliary junction (APBJ).
- With APBJ, the pancreatic duct and biliary tree fuse to forma common channel before passage through the duodenal wall.
- The fused duct forms a long common channel, which allows pancreatic secretions to reflux into the biliary tree. Because the pancreatic duct has higher secretory pressures than the biliary tree, exocrine pancreatic secretions reflux up into the bile duct and can inflame and damage the biliary tree, resulting in cystic degeneration.
How to classify choledochal cyst. [TU 2072/3]
Classify choledochal Cysts .Briefly describe their clinical features and management. [TU 2069]
Classify Choledochal cyst with illustrations. Discuss the management of choledochal cyst according to the type. [TU 2073]
Short note on Choledochal cyst. [TU 2066/1]
Todani Classification of Choledochal cysts?
Type I - involves only the extrahepatic biliary tree, generally a fusiform dilation, most common type
Type II - saccular diverticulum off the common bile duct, may be mistaken for an accessory gallbladder.
Type III - cystic dilation of the intramural common bile duct, within the wall of the duodenum (choledochoceles)
- IVa - Cysts involving the intrahepatic and extrahepaticbiliary tree
- IVb - Multiple cysts limited to the extrahepatic biliary tree.
Type V (Caroli disease) - intrahepatic ducts, may be solitary but usually occur diffusely in all segments
Clinical features of choledochal cyst?
- The classic triad of jaundice, right upper quadrant pain, and a palpable mass occurs rarely.
- Nausea, pruritus, and weight loss. Long standing disease can induce a chronic injury to the liver with cirrhosis.
- Cholangitis, pancreatitis, hepatic fibrosis, and malignant disease have all been reported at the time of presentation.
- An unusual presentation is that of acute rupture of the cyst, with subsequent bile peritonitis.
Discuss management of type I choledochal cyst. [TU 2072/3]
Treatment of choledochol cyst?
Type I - complete surgical excision, cholecystectomy,and Roux-en-Y hepaticojejunostomy. The proximal extent of resection should continue to the nondilated biliary tree and may require anastomosis to the left and right hepatic ducts. The distal duct is oversewn, with care taken not to injure the pancreatic duct If dissection of a cyst from the portal vein or hepatic artery is technically difficult, some surgeons advocate leaving the posterior cyst wall intact and performing a mucosectomy. The mucosectomy is performed in an attempt to decrease the risk of malignancy.
Type II - complete excision and in the presence of an APBJ, Rouxen-Y hepaticojejunostomy
Type III cysts - may be approached transduodenally. Because the pathogenesis of type III cysts is not clear and may not involve APBJ, endoscopic drainage may suffice. In the setting of duodenal or biliary obstruction, transduodenal excision or sphincteroplasty can be performed.
Type IV cysts affecting only the extrahepatic bile ducts are managed similarly to type I cysts. Those with intrahepatic extension involving only one lobe can be treated with partial hepatectomy and reconstruction.
Caroli disease - resection if the disease is unilobar to liver transplantation when diffuse disease is detected.
If there is anomalous pancreaticobiliary duct junction while excising the lower limit of the cyst the injury to the anomalous opening of the pancreatic duct should be avoided.
Recurrent attacks of cholangitis can lead to gross pericholecystic adhesion and the cyst adhesion to the hepatic artery or the portal vein. In such situation, modified approach is required to remove such cyst. The anterior and lateral wall of the cyst is excised. An arbitrary plane is created within the posterior wall of the cyst and the inner lining of the cyst is dissected and excised leaving behind the outer layer of the cyst that lies adherent to the portal structures. Thus, the anterior, lateral and the inner lining of the posterior wall of the cyst is excised. This is called Lily’s modification for excision of choledochal cyst.
Why a cystoenterostomy is not an ideal surgical procedure?
The mucus lining of the choledochal cyst shows inflammation and fibrosis—so there is chance of anastomotic obstruction from scarring at the site of anastomosis.
The fibrous thick walled cyst does not contract after drainage and acts as a receptacle for stagnant bile and the risk of cholangitis remains.
Choledochal cyst is a premalignat lesion and the chance of developing malignancy remains
Classification of Biliary Atresia
- Type I – Atresia restricted to CBD
- Type II – Atresia of CHD
- Type III – Atresia of left and right Hepatic duct
Treatment of Biliary Atresia
For type II and III – Kasai Operation – Excision of biliary tract with Roux-en-Y loop of jejunum anastomosis with exposed area of liver capsule, above the bifurcation of portal vein
Management of GB polyp?
For polyp >1.8cm – Extended cholecystectomy
Gall stones are independent risk factors for GB carcinoma. So, all polyp with Gall stones require cholecystectomy.
Etiological risk factors for carcinoma of gallbladder?
- 1. Gallstone disease.
- 2. Choledochal cyst.
- 3. Anomalous pancreaticobiliary duct junction.
- 4. Gall bladder polyp >1 cm.
- 5. Adenomyomatosis of gallbladder.
- 6. Chronic typhoid carriers
- 7. Carcinogenes, e.g. nitrosamines.
Clinical features of gall bladder carcinoma?
- • Symptoms and signs suggestive of acute cholecystitis
- • Symptoms and signs of chronic biliary tract disease—right upper quadrant pain, jaundice
- • General symptoms and signs suggestive of a malignant disease—anorexia, weight loss,
- generalized weakness
- • Symptoms and signs suggestive of disease outside the biliary tract—gastric outlet obstruction and gastrointestinal bleeding
- • Symptoms and signs suggestive of advanced malignant disease—palpable gallbladder
- mass, hard nodular liver and ascites.
How to stage the carcinoma of gall bladder? [TU 2073]
Mention stages of GB carcinoma and describe its management according to its stage. [TU 2068/2]
TNM Classification of Gall Bladder?
- T1 Tumor invades lamina propria or muscle layer
- T1a Tumor invades lamina propria
- T1b Tumor invades muscle layer
- T2 Tumor invades perimuscular connective tissue; no extension beyond serosa or into liver
- T3 Tumor perforates the serosa (visceral peritoneum) and/or directly invades the liver and/or one other adjacent organ
- T4 Tumor invades main portal vein or hepatic artery or invades two or more extrahepatic organs
- N1 - Node along cystic duct, CBD, Hepatic artery, or portal vein
- N2 - Periaortic, pericaval, SMA, Celiac artery nodes
M1 Distant metastasis
Draining nodal basin for gallbladder cancer?
- Hepatoduodenal ligament.
- From there, affected lymph nodes may include periaortic nodes near the celiac axis or pancreaticoduodenal nodes around the superior mesenteric artery.
- Because the venous drainage of the gallbladder includes direct venous tributaries into the liver parenchyma, these tumors may spread directly into segment IV of the liver.
- Transperitoneal spread is alsocommon and can progress to carcinomatosis
Discuss the management of incidental carcinoma of gall bladder. [TU 2073]
Treatment of Ca GB
- A. Patients with incidental findings
- 1. Gallbladder polyp > 10 mm - open cholecystectomy
- 2. Gallbladder cancer after cholecystectomy
- T1a - Cholecystectomy
- T1b with margin negative - Cholecystectomy is sufficient
- T1b lesions with perineural, lymphatic, or vascular invasion - extended cholecystectomy
- T2 - Extended Cholecystectomy
Because gallbladder cancer is generally unresponsive to other therapies, the presence of any residual disease after operative intervention predicts poor outcome.
- B. Patients suspected of having gallbladder cancer preoperatively.
- - diagnostic laparoscopy
- - T3 and T4 - Radical Cholecystectomy
- C. Patients with advanced disease at presentation - Paliation
- Jaundice - endoscopic biliary stenting, and self-expanding endobiliary metal stents
- Pain - oral narcotics, parenteral opioids
- Percutaneous neurolysis of the celiac ganglion
What is extended cholecystectomy?
- Resection of 2 cm of apparently normal hepatic parenchyma from the gallbladder fossa
- Removal of the pericholedochal, periportal, hepatoduodenal, right celiac, and posterior pancreaticoduodenal lymph nodes
- Resection of the cystic duct margin to uninvolved mucosa may require resection of the common bile duct with Roux-en-Y reconstruction.
- All port sites should also be excised.
Kocherization of duodenum followed by frozen section of aortocaval lymph nodes should be done before extended cholecystectomy. Only if the node is negative, we can proceed for extended cholecystectomy.
What is Radical cystectomy?
- Radical Cholecystectomy, includes at least segments IVB and V but more often requires a central hepatectomy including all of segments IV, V, and VIII.
- Right trisegmentectomy (removal of IV,V,VI,VII,VIII) may be used.
(Note that gallbladder fossa is the anatomical demarcating line between the right and left lobes and carcinoma gall bladder may spread to both lobes)
Etiological factors for cholangiocarcinoma?
- Stone disease
- Bacterial induced endogenous carcinogenes in the bile
- Sclerosing cholangitis and ulcerative colitis
- Choledochal cyst
- Parasitic infestation of bile duct - Clonorchis sinensis
Types of cholangiocarcinoma?
- A. Depending on the sites of involvement
- 1. Intrahepatic
- 2. Perihilar 3. Distal
- Klatskin’s tumor - involving the confluence of the hepatic ducts.
- According to the UICC classification, Perihilar cholangiocarcinoma is proximally separated from intrahepatic cholangiocarcinoma by the second-order bile ducts, and is distally separated from distal extrahepatic cholangiocarcinoma by the insertion of the cystic duct into the extrahepatic biliary tree.
- B. Depending on the gross appearance:
- • Scirrhous - diffuse thickening of wall of the bile duct. Intensely fibrotic lesion
- • Nodular: extraductal nodule in addition to intraluminal projection.
- • Papillary : Mainly involves the distal bile duct and the periampullary region. Friable vascular growth may fill the bile duct lumen and bleeds easily leading to hemobilia. [@ PaNoS]
Bismuth-Corlette classification for perihilar cholangiocarcinomas?
- Type I - limited to the common hepatic duct, below the level of the confluence of the right and left hepatic ducts.
- Type II - involves the confluence of the right and left hepatic ducts.
- Type IIIa - type II and extends to the bifurcation of the right hepatic duct.
- Type IIIb - type II and extends to the bifurcation of the left hepatic duct
- Type IV - extending to the bifurcations of both right and left hepatic ducts or multifocal involvement
- Type V - stricture at the junction of common bile duct and cystic duct
TNM Intraheptaic Bile duct Tumor
T1 - Soliary Tumor without vascular invasion
T2a - Solitary tumor with vascular invasion
T2b - Multiple tumor with/without vascular invasion
T3 - Tumor perforating the visceral peritoneum or involving extrahepatic structure by direct extension
T4 - Tumor with periductal invasion
TNM Perihilar Bile duct Tumor
T1 - Tumor Confined to bile duct, with extension upto muscular layer or fibrous tissue
T2a - Tumor invading beyond the wall of bile duct to surrounding adipose tissue
T2b - Tumor invades adjacant Hepatic parenchyma
T3 - Tumor invades unilateral branches of portal vein or hepatic artery
T4 - Tumor invades main portal vein or its branches bilaterally or CHA or the secondaries, biliary radicals b/l or, Unilateral second order biliary radical with contralateral portal vein or hepatic artery involved.
Blumgart classification of hilar carcinoma?
- T1 –
- Hepatic biliary confluence and/or
- one secondary biliary duct
- T2 –
- Hepatic biliary confluence and/or
- one secondary biliary duct, complicated with the involvement of the homolateral portal vein and/or homolateral liver lobe atrophy
- T3 –
- hepatic biliary confluence and
- both secondary biliary ducts, or
- one secondary biliary duct, with involvement of contralateral portal vein, or
- both secondary biliary ducts, with contralateral liver lobe atrophy,
- involvement of main portal vein or
- involvement of bilateral portal veins
Criteria for non-resectability in cholangiocarcinoma?
- Medical contraindications
- Advanced cirrhosis/Portal HTN
- Main portal vein involvement
- B/l second order biliary radical involvement
- Lobar atrophy with contralateral second order radical involvement
- Lobar atrophy with contralateral portal vein involvement
- N2 nodal involvement (periaortic, periportal)
- Distant metastasis
Describe management options of hilar cholangioca with dissociation of right and left hepatic ducts. [TU 2067/2]
Management of cholnagiocarcinoma?
Hilar Cholangiocarcinoma -
Type I and II - Bile duct exicision with 5-10mm of resection with cholecystectomy with HJ (frozen section to confirm resection margin)
Type II requires resection of caudate lobe
Type III and IV - Complex resection and reconstruction of portal vein, hepatic artery or both.
Distal Cholangiocarcinoma - Whipple's procedure
TNM of ampullary carcinoma
- T1 - limited to ampulla of vater of sphincter of oddi
- T2 - Duodenal wall
- T3 - Pancreas
- T4 - Peripancreatic soft tissue or other adjacant structures other than pancreas
N1 - regional LN metastasis
Biopsy in cholangiocarcinoma?
- If resectable - donot take biopsy
- If non-resectable - take biopsy
In periampullary carcinoma, do not wait for biopsy reports for operative procedure.
A thirty five years male patient came ot ER with the complains of severe abdominal pain since two hours. He has nausea , mild yellowish eyes ,severe colicky pain without fever. His bowel and bladder habits are normal, however he had history of alcohol intake previously . What could be his problem of suffering from? How do you reach into the diagnosis? Mention your line of emergency management . 73