1. Approaches to Difficult ERCP Cannulation, Part 1 of 3 Kaveh Mojtahed, MD GI Fellow

2. The biliary tree and most things internal medicine doctor need to know Kaveh Mojtahed, MD

3. Objectives Common terminology Gallstone diseases ERCP indications and complications Brief review of pancreatic cysts Biliary malignancies Topics not covered: biliary cysts, chronic gallbladder dysfunction, biliary atresia, gallbladder polyps, HIV cholangiopathy

4. Chole-what Cholelithiasis Cholecystitis Choledocholithiasis Cholangitis Cholecystectomy Cholangiocarcinoma

8. Charcot’s triad vs Raynaud’s Pentad Fever RUQ pain Jaundice Hypotension Altered mental status Only 50-70% develop all Charcot’s triad

9. Case #1 34 year old male presents to general clinic with episodes of severe epigastric and RUQ abdominal pain

11. H&P Starts 30 minutes after eating, lasts for 4 hrs, then resolves, refers to scapula and right upper back Exam: anicteric sclera, no Murphy’s sign

12. Jaundice Scleral icterus and sublingual, total bili 2-2.5 Cutaneous jaundice, total bili 5 Tympanic membrane, total bili 10 Hemolysis does not increase total bili > 5 Clay-colored stools = obstructive jaundice Occult blood in clay colored stool suggests pancreatic or ampullary CA

13. Murphy’s sign 65% sensitivity, 88% specificity Pain and arrested inspiration when the examiners fingers are hooked under right costal margin at mid- clavicular line

14. Labs/imaging CBC and LFTs- normal

15. What’s the diagnosis? Biliary colic He decides to defer cholecystectomy for now

16. Is this a good idea? ~60% of symptomatic gallstone patients continue to have symptoms 90% of complications (eg cholecystitis) preceded by uncomplicated biliary colic

17. 3 months later Constant RUQ pain for 24 hrs T 39, HR 105, BP 110/53 Exam: + Murphy’s WBC 15, Hgb 15, Plt 210, INR 1.1 AST 120, ALT 145, AlkP 290, Total bili 4.9, Lipase 200

18. Differential What disease process is occuring? Acute cholecystitis Cholangitis Gallstone pancreatitis Choledocholithiasis Biliary Colic

21. Cholangitis Early antibiotic use Biliary decompression **Elderly, diabetics, immunocompromised do not have typical presentation**

22. Antibiotic coverage: 3 most common GN bacteria implicated in cholangitis? E. Coli Klebsiella Enterobacter What is the most common GP bacteria Enterococcus Anaerobes

23. Antibiotics GNR- ampicillin/sulbactam, piperacillin- tazobactam, ceftriaxone, levofloxacin, ciprofloxacin, carbapenems Anaerobes- Zosyn/Unasyn, metronidazole

24. Biliary decompression Urgent ERCP 5, age >75, etc* (refer Tokyo 2013 guidelines)] Early ERCP <72hrs with mild cholangitis responding to medical therapy Cholecystectomy once clinically stable

25. ERCP Endoscopic retrograde cholangiopancreatography Indications: stone disease, malignancy, stricture, recurrent/chronic pancreatitis Contraindications: abnormal anatomy, pancreatitis (unless need to remove gallstone) What’s an esophageal abnormality that would be a high risk situation for perforation with passing a side viewing scope?

26. Zenker’s diverticulum

29. Successful stone extraction

30. Post-ERCP patient starts to eat and develops severe epigastric pain Lipase is 1900

31. What are the main complications of ERCP? Perforation (esophageal/duodenal/biliary) Post-ERCP pancreatitis (2-10%) - costs healthcare system $150 million/year Post-sphincterotomy bleed

32. How do you diagnose post-ERCP pancreatitis? 1.New or increased abdominal pain 2.Pancreatic enzymes 3x ULN 24 hrs post ERCP 3.Resultant hospitalization more than a night RF: any injection, probing or manipulation of pancreas or its duct, sphincterotomy

33. Reducing post-ERCP pancreatitis Prophylactic pancreatic STENT placement (18 trials have shown reduces risk of PEP by 70%, NNT 8) PR INDOMETHACIN immediately after procedure (meta-analysis of 912 pateints, 64% reduction in PEP)

34. A few other important things Acalculous cholecystitis Risk factors: sepsis, TPN, prolonged fasting, sickle cell disease, Salmonella infections, diabetes mellitus, cytomegalovirus, cryptosporidiosis, microsporidiosis Antibiotics, percutaneous drain, cholecystectomy

35. HIDA

36. Gallstone disease key points Asymptomatic gallstone disease has a benign course and can be managed with observation. Biliary colic is the most common clinical presentation in patients with symptomatic gallstones. Laparoscopic cholecystectomy is the treatment of choice for biliary colic and acute cholecystitis.

37. A few other biliary diseases 1. Spinchter of Oddi dysfunction 2. Recurrent pyogenic cholangitis 3. Primary sclerosing cholangitis

38. Spinchter of Oddi dysfunction Manometry Nifedipine for Type 3 and mild 2 ERCP for Type 1 with spinchterotomy

39. Recurrent pyogenic cholangitis Intrabiliary pigment stone formation resulting in stricture and obstruction leading to recurrent cholangitis Stone formation thought to be instigated by parasite (Clonorchis sinesis) or bacterial infection Exclusively SE Asians

40. https://www.youtube.com/watch?v=g 18B2rm78E4

41. PSC intra/extrahepatic bile duct inflammation/fibrosis Alk phos 3-5 x ULN Ulcerative colitis ERCP/MRCP Cholangiocarcinoma Treatment: Ursodeoxycholic acid 13-15 mg/kg/day- no change in survival but improves LFTs

42. Demographic lesson Who gets PSC? middle aged men, 70% of PSC patients are men average age 40 Who gets PBC? middle aged woman, 10 times more than men. Incidence in US women 1/1000 over age 45

43. Pancreatic cysts

44. Complete list of pancreatic cysts Widespread use of CT and MRI = 13.5% prevalence of incidental cysts Epidermoid Cyst in Intrapancreatic Spleen Intraductal Oncocytic Papillary Neoplasm 1. Intraductal Papillary Mucinous Neoplasm (IPMN) Intraductal Tubular Adenoma Intraductal Tubular Carcinoma Lymphoepithelial Cyst Mucinous Cystic Neoplasm Pancreatic Intraepithelial Neoplasia Paraduodenal Wall Cyst 2. Pseudocyst Serous Cystadenocarcinoma Serous Macrocystic / Oligocystic Adenoma Serous Microcystic Adenoma Solid and Cystic Hamartoma of the Pancreas Solid Pseudopapillary Neoplasm Solid Serous Adenoma Squamoid Cyst of Pancreatic Ducts von Hippel Lindau Pancreatic Lesions

45. IPMN Main vs side branch intrapapillary mucinous neoplasm Risk of carcinoma 70% in main branch IPMN >3 cm Recurrent pancreatitis Increased risk of extra-pancreatic malignancies

47. Pseudocyst or “walled off pancreatic fluid collection” Non-epithelial lined lesion formed from resorption of fat necrosis Pseudoaneursym 40% resolve on their own If symptomatic can undergo drainage procedure

49. Biliary malignancies Cholangiocarcinoma Ampullary adenocarcinoma

50. Cholangiocarcinoma Risk factors: PSC, biliary atresia, chronic infection with liver flukes, and biliary cysts 60-70%- Klatskin tumor or more distal = complete obstruction Symptoms: painless jaundice, right upper quadrant pain, and weight loss CA 19-9, CEA, AFP MRCP/ERCP

51. Ampullary adenocarcinoma familial adenomatous polyposis or Peutz- Jeghers syndrome pancreaticoduodenectomy (Whipple procedure)

52. Summary Common terminology Gallstone diseases ERCP indications and complications Brief review of pancreatic cysts Biliary malignancies

53. Question 85 year old diabetic male in ER for 2 days confusion and poor appetite. He is cool, clammy, no fever, BP 90/70, HR 110, RR 32, nontender abdomen. WBC 7, ALKP 550, ALT 120, AST 190, Total bili 3, U/S normal liver, gallstones present, no duct dilatation.

54. What’s the next step in management? A) HIDA B) ERCP C) MRCP D) cholecystectomy

55. Key point In severely ill patients with hypotension and sepsis and a high clinical suspicion for acute cholangitis with or without confirmatory imaging studies Preferred next diagnostic test is ERCP Diabetics and elderly do not have typical presentations!