Benign biliary disease and biliary injury- biliary physiology, pathology, presentation and management

1. Benign biliary disease and biliary injury- biliary physiology, pathology, presentation and management Claire Edwards 09/09/09

3. Biliary anatomy

4. Biliary anatomy Cystic artery is usually a branch of the R hepatic

5. Biliary histology Layers of gallbladder: Epithelium (columnar) Lamina propria Smooth muscle Subserosal connective tissue Serosa No submucosa or muscularis mucosa! CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi

6. Biliary physiology Liver produces 0.5 to 1.0 L bile/day Vagal input, GI hormones (cholecystokinin, secretin, gastrin) increase bile production GB epithelium absorbs sodium and water, secretes mucin and acid. CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi. Bile in GB is concentrated relative to bile from liver. Active sodium transport, passive water absorption CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi. Bile in GB is concentrated relative to bile from liver. Active sodium transport, passive water absorption

7. Biliary physiology Contents of bile: water, electrolytes, bile salts, proteins, lipids, bile pigments Primary bile salts: cholate, chenodeoxycholate Conjugated w/ glycine, taurine in liver to become bile acids 95% of bile acids are re-absorbed via enterohepatic circulation Cholesterol crystals, mucin are nucleating agents for stones. Decreased stability of cholesterol-containing vesicles

8. Jaundice  Conjugated (direct) bili? increased in biliary obstruction Unconjugated (indirect) bili? increased in hemolysis, hepatocellar injury Scleral icterus at >2.5 mg/dL Jaundice at > 5 mg/dL

9. Gallstones  Of those with gallstones, 1% per year develop serious symptoms or complications and need cholecystectomy. Risk factors: Crohn’s disease, terminal ileal resection, TPN/fasting States of excess cholesterol contribute to cholesterol gallstones

10. Gallstones  Pigment stones Dark 2/2 calcium bilirubinate Black pigment stones: hemolytic states, cirrhosis Brown pigment stones Within bile ducts Associated with infection, biliary stasis

11. Gallstones  Biliary colic Only assoc. w/ meals in 50% Pain attack lasting > 24 hrs? acute rather than chronic cholecystitis Biliary colic, no stones, sludge detected on more than one occasion ? lap chole Biliary symptoms may also be caused by cholesterolosis, polyps (see later)

12. Cholecystitis  Presentation: N & V, pain, fever, persistent (unlike colic) RUQ or epigastric pain, Acute cholecystitis—exacerbated by touch: Murphy’s sign is due to peritoneal irritation. Most cases? gallstone dislodges, inflammation resolves Or may lead to inflammation and necrosis of the GB wall (gangrenous cholecystitis)? GB abscess Secondary infection w/ gas forming organisms? emphysematous cholecystitis

13. Acute Cholecystitis Management: early cholecystectomy (2-3 days) Morbidity, length of stay, return to work all better w/ laparoscopic chole Predictors of conversion to open: older age, higher ASA, male gender, thickened GB wall, obesity GB empyema, perforation, emphysematous cholecystitis? need emergency cholecystectomy If cannot see to ligate cystic duct 2/2 inflammation? partial cholecystectomy, cauterization of remaining GB mucosa, drainage OR if pt too unstable for OR? cholecystostomy tube, IV abx, then lap chole in 3 months

14. Choledocholithiasis Primary stones: biliary stasis (stricture, papillary stenosis, tumors, secondary stones), biliary infection Secondary calculi: started in GB, then migrated. “Retained” if discovered < 2 years after cholecystectomy (1-2%) “Recurrent” if discovered > 2 yrs after cholecystectomy Dx: consider MRCP; ERCP is potentially therapeutic as well as diagnostic Complete endoscopic clearance of common duct stones: 75% of pts w/ one procedure, 90% w/ multiple procedures.

15. Imaging Ultrasound: >98% sensitive, > 95% specific for stones Cholecystitis: ductal dilation, pericholecystic fluid, wall thickening, sonographic Murphy’s sign Common bile duct? upper limit of nl is 7 mm Extrahepatic ducts > 10 mm or intrahepatic ducts > 4 mm suggests obstruction GB wall is thickened if > 4 mm. Mass lesion evidence: stone that does not move when patient does, asymmetric thickening

16. Imaging KUB shows 15% of stones (only show up if calcified) CT Cholangiography: Noninvasive. IV contrast, excreted in biliary system. Good for ductal anatomy. Only about 60% sensitive for stones (only show up if calcified) MRC: Good for common bile duct stones PTC: Used instead of ERCP for proximal stones.

17. Imaging HIDA: Cystic duct obstruction is present if GB not visualized 2 hours after injection Biliary dyskinesia: Ejection fraction less than 35% at 20 minutes after CCK administration is considered abnormal. Nl HIDA excludes acute cholecystitis. High false positive rate when pt is fasting (passage of tracer slows)

18. Normal HIDA scan

19. Abnormal HIDA scan

20. Other indications for cholecystectomy Gallstone pancreatitis Remove GB same admission Biliary dyskinesia

21. Lap chole Conversion rate should be 5%. In acute cholecystitis may be up to 30%. 2nd trimester in pregnant women CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi CCK: released from duodenum when intraluminal fat, amino acids, and gastric acid are present; also causes relaxation of the Sphincter of Oddi

22. Laparoscopic cholecystectomy: critical view

23. Postcholecystectomy pain With jaundice, biloma, or significant pain? ERCP looking for retained stone or CBD injury or stricture

24. Complications of cholecystectomy Bile duct injury 25% are recognized intraoperatively Primary repair over T-tube if small, nonthermal injury Otherwise, choledochojejunostomy If recognized post-op AND in the presence of leak, wait 6 weeks (too much inflammation) Biliary stricture If returning to OR, success is increased by getting a percutaneous or ERCP cholangiogram preop Biliary-enteric bypass more successful than primary end-to-end biliary repair Ischemic biliary strictures will not respond permanently to IR/endoscopic dilation Remember drains, referral to tertiary center is an option Remember drains, referral to tertiary center is an option

25. Complications of cholecystectomy Biliary leak From cystic duct stump (acute cholecystitis inflammation can make clips come off) or duct of Luschka Widely drain and do endoscopic stenting, too much inflammation in presence of bile leak to re-repair

26. Complications of cholecystectomy Bismuth classification of biliary injuries: Type I: > 2 cm below hepatic duct bifurcation Type 2: < 2 cm Type 3: hepatic duct confluence is intact but no residual common hepatic duct exists Type 4: destruction of hilar confluence, L and R hepatic ducts are completely separated Type 5: involve branch of right hepatic duct +/- the common bile duct Bismuth classification determines whether you will have to do individual hepaticojejunostomies of the left and right ducts Bismuth classification determines whether you will have to do individual hepaticojejunostomies of the left and right ducts

27. Gallstone ileus “Tumbling obstruction” Terminal ileum? most common site of obstruction due to narrow lumen Enterotomy, milk stone proximally and out Make sure bowel does not need to be resected in the area where the stone was impacted Closure of biliary-enteric fistula and cholecystectomy (at second laparotomy if there is too much RUQ inflammation)

28. CBD exploration Perform choledochotomy Leave 16 Fr or larger T-tube in place. Follow-up cholangiogram Clamp tube and pull in 4-6 weeks if no more stones If stones still present, removal via T tube in 4-6 weeks Or transduodenal sphincterotomy (if stone impacted at ampulla) Lap CBD exploration: Transcystic approach vs. choledochotomy approach saline irrigation, stone basket or choledochoscope via cystic duct Larger stones need choledochotomy approach. CBD needs to be at least 6 mm Need T-tube Need drainage procedure when stones cannot be cleared or duct is > 1.5 cm in diameter. Sphincterotomy: incise sphincter at 11:00 position to avoid pancreatic duct. Drainage procedure: choledochoduodenostomy or choledochojejunostomy, depending on ability to mobilize the duodenum and tension on the anastomosis. Duodenal anastomosis can result in sump syndrome (obstruction by food) Sphincterotomy: incise sphincter at 11:00 position to avoid pancreatic duct. Drainage procedure: choledochoduodenostomy or choledochojejunostomy, depending on ability to mobilize the duodenum and tension on the anastomosis. Duodenal anastomosis can result in sump syndrome (obstruction by food)

29. Acute acalculous cholecystitis More fulminant course than calculous cholecystitis Possibly related to visceral ischemia, gallbladder stasis Risk factors: elderly, TPN, major abdominal surgery (AAA) U/S is test of choice. HIDA has high false (+) rate. Percutaneous cholecystostomy in those who cannot go to OR (90% improve)

30. Acute cholangitis Have to have obstruction (usu. common duct stone) Must do cholangiography If very sick/septic needs urgent biliary decompression/relief of obstruction? PTC or ERCP

31. Recurrent pyogenic cholangitis Intrahepatic stones from infection w/ biliary bacteria or parasites (Clonorchis sinensis, Opisthoricis, Ascaris) Asian population Recurrent episodes of cholangitis due to partial obstruction Possibly hepatic abscesses, cirrhosis Risk for cholangiocarcinoma Roux-en-Y hepaticojejunostomy w/subcutaneous afferent (Hudson) loop for endoscopic access Extended hepatectomy of dominant stone-containing lobe, if there is one, for cholangiocarcinoma risk

32. Primary sclerosing cholangitis Intra- and extrahepatic biliary strictures 60-70% have ulcerative colitis 1% per year risk of cholangiocarcinoma Endoscopic/percutaneous brushings of strictures for surveillance Jaundice, pruritis, fatigue 10-12 years average lifespan from time of diagnosis DX: cholangiography If end-stage liver disease? transplant. 85% 5 year survival Medical therapy: “disappointing,” ursodeoxycholate only improves numbers, not patient Rapid onset of jaundice, CA 19-9 > 100 are helpful but not predictive in cholangiocarcinoma Rapid onset of jaundice, CA 19-9 > 100 are helpful but not predictive in cholangiocarcinoma

33. Benign biliary strictures Multiple causes: pancreatitis, cholangitis, etc. Anastomotic strictures: Have to do PTC to dilate biliary-enteric anastomosis because difficult to access Roux en Y endoscopically.

34. Choledochal cysts Anomalous biliary duct-pancreatic duct junction with long common channel, reflux of pancreatic enzymes, and cystic breakdown of biliary tract 10 % have classic triad of RUQ pain, jaundice, palpable mass Severe possible complications: cirrhosis, portal hypertension, cyst rupture w/ bile peritonitis GB, liver, or cholangiocarcinoma incidence? 3 to 25% U/S, CT scan to diagnose; cholangiography to plan operative treatment

35. Choledochal cysts Type I: Extrahepatic Type II: diverticular extrahepatic Therapy for Type I and II cysts: cholecystectomy, resection of extrahepatic bile ducts w/ hepaticojejunostomy TypeIII: Intraduodenal Type IVa: extrahepatic and intrahepatic Type IV b: multiple sections of extrahepatic bile ducts Tx: resect extrahepatic cyst Type V: Caroli’s disease. Intrahepatic ducts only. Do liver resection of involved area if possible. Liver transplantation Type I: Extrahepatic Type II: diverticular extrahepatic Therapy for Type I and II cysts: cholecystectomy, resection of extrahepatic bile ducts w/ hepaticojejunostomy TypeIII: Intraduodenal Type IVa: extrahepatic and intrahepatic Type IV b: multiple sections of extrahepatic bile ducts Tx: resect extrahepatic cyst Type V: Caroli’s disease. Intrahepatic ducts only. Do liver resection of involved area if possible. Liver transplantation

36. Biliary fistulas Cholecystoenteric fistula From gallstone eroding into viscus: #1 duodenal, #2 colon Mirizzi’s syndrome: Stone impacted in neck of GB or in cystic duct? inflammation? hepatic duct obstruction? jaundice From PUD: choledochoduodenal or choledochogastric fistula Rarely, from malignancy or trauma

37. Polypoid lesions of the gallbladder Cholesterolosis: deposits of cholesterol in GB wall Cholesterol polyp: pedunculated, epithelialized focus of cholesterolosis No shadow on U/S Adenomatous hyperplasia: increased thickness of mucosa and muscle Mass lesion on U/S Adenomatous polyp: true neoplasm Granular cell myoblastoma: benign tumor of neuroectoderm, needs cholecystectomy Symptomatic? cholecystectomy Suspicion of carcinoma (e.g., invasion by U/S)? open cholecystectomy Otherwise if less than 1 cm, observe. Fibroxanthogranulomatous inflammation: Foamy histiocytes/inflammatory cells/fibroblastic vascular rxn/mucosal ulcerationSymptomatic? cholecystectomy Suspicion of carcinoma (e.g., invasion by U/S)? open cholecystectomy Otherwise if less than 1 cm, observe. Fibroxanthogranulomatous inflammation: Foamy histiocytes/inflammatory cells/fibroblastic vascular rxn/mucosal ulceration

38. Benign biliary lesions Papilloma or adenoma of bile duct Often jaundice b/c often close to ampulla Resect lesion w/ some duct wall (to avoid recurrence) Transduodenally for ampullary lesions Benign inflammatory tumors: pseudotumors Usually extrahepatic, but above biliary bifurcation.

39. References Townsend, C.M., ed. 2008. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice, 18th ed. Philadelphia: Saunders Elsevier. Mulholland, M., ed. 2006. Greenfield’s Surgery: Scientific Principles and Practice, 4th ed. Philadelphia: Lippincott Williams and Wilkins. HIDA scan images downloaded from “Emedicine: Cholecystitis: Multimedia.” http://emedicine.medscape.com/article/171886-media Bines, S.D., et.al. 2007. Rush University Medical Center Review of Surgery. Philadelphia: Saunders Elsevier.