1. The document discusses several diseases of the pancreas including congenital problems like annular pancreas and pancreas divisum. 2. It covers acute and chronic pancreatitis, their causes, symptoms, prognosis, and treatment options including supportive care and surgery. 3. The document also discusses several types of pancreatic tumors and cysts, their diagnosis and treatment through surgery or other palliative options. It provides details on specific neuroendocrine tumors.

1. Diseases of the Pancreas John Christein Section of Gastrointestinal Surgery Department of Surgery

2. Congenital Problems

3. Annular Pancreas Developmental malrotation of the dorsal and ventral pancreas Resulting in duodenal narrowing or obstruction 50% identified in childhood Treatment: duodenal bypass; do not divide pancreas - leak, pancreatitis

4. Annular Pancreas

5. Pancreas Divisum Non-fusion of the ducts of Wirsung and Santorini Maintain separate openings into duodenum 3-10% population Rediscovered with widespread use of ERCP/EUS Rare cause of recurrent acute pancreatitis Surgical therapy: sphincteroplasty of duct of Santorini plus/minus Wirsung

6. Pancreas Divisum

7. Pancreas Divisum Recurrent acute pancreatitis Endoscopic minor and major sphincteroplasty Transduodenal minor sphincteroplasty

8. Pancreatitis

9. Marseilles Classification Acute pancreatitis Recurrent acute Recurrent chronic Chronic pancreatitis

10. Acute Pancreatitis Moynihan 1925: “Acute pancreatitis is the most terrible of all calamities that occur in connection with the abdominal viscera. The suddenness of its onset, the illimitable agony which accompanies it and the morality attendant upon it render it the most formidable of catastrophes.”

11. Acute Pancreatitis Pathologic classification Edematous (mild) 80-90% Hemorrhagic Necrotizing

12. Acute Pancreatitis Pathophysiology Not clearly defined Parenchymal or ductal disruption/obstruction Reflux of bile/duodenal contents into the duct Increased ductal permeability Activation of pancreatic enzymes (trypsinogen) Compliment activation Ischemic injury Reperfusion injury

13. Acute Pancreatitis Morality Overall < 2% Severe pancreatitis 15% (>3 Ranson’s criteria)

14. Acute Pancreatitis Etiology Biliary disease, ethanol > 80% Other main causes Hyperlipidemia (Types I, IV, V) Pancreas divisum Idiopathic ERCP Other causes: peptic ulcer, trauma, drugs (thiazides, steroids, imuran), hypercalcemia, hereditary, infectious agents (viral, bacterial), pancreatic carcinoma

15. Acute Pancreatitis Diagnosis Symptoms: progressive epigastric pain, nausea, vomiting Signs: hypovolemia, tachycardia, orthostasis, abdominal tenderness/fullness Laboratory studies: amylase, lipase, LFT

16. Acute Pancreatitis Imaging Plain films: segmental GI ileus Ultrasound: pancreatic edema CT scan: phlegmon, edema, fluid collections Dynamic CT scan: bolus IV contrast. Defines areas of hypoperfusion/peripancreatic necrosis

17. Acute Pancreatitis Differential diagnosis Perforated peptic ulcer Mesenteric ischemia (acute) Strangulated small bowel Biliary colic Myocardial infarction

18. Acute Pancreatitis Prognostic factors-Ranson’s criteria On admission: Age over 55 years WBC count > 16,000 Blood glucose > 200 Serum LDG > 350 Serum SGOT > 250

19. Acute Pancreatitis Prognostic factors-Ranson’s criteria After 48 hours: Hematocrit fall > 10% BUN rise > 5 Calcium <8 pO2 < 60 Base deficit > 4 6 liters or more fluid sequestration No Risk Factors Mortality (%) < 3 1 3-4 15 5-6 40 7-8 100

20. Acute Pancreatitis Supportive therapy Volume resuscitation: CVP, Swan-Ganz Analgesia Antibiotics: No role in mild pancreatitis May decrease sepsis rate in severe NECROTIZING pancreatitis, but offers no survival advantage

21. Acute Pancreatitis Therapy Supportive therapy Post duodenal enteral feeding TPN: does not alter course of disease. Helps maintain nutrition Endoscopic sphincterotomy if an impacted gallstone is suspected

22. Acute Pancreatitis Operative Indications Clinical deterioration Infected Necrosis Operative procedure Drain & debride necrotic/infected tissue

23. Acute Pancreatitis Complications Pancreatic/peripancreatic Infected Necrosis Translocation of gram negative organisms from the GI tract. Diagnosis: septic clinical course and air on CT/ Percutaneous aspiration for culture. Treatment: Percutaneous drainage, well defined, loculated, liquefied abscesses Surgical debridement/draining Mortality 50%

24. Pseudocyst Walled off area of pancreatic necrosis and/or juice 60-80% resolve spontaneously Diagnosis: US, CT Operation only if Infection, hemorrhage duodenal and/or biliary obstruction PAIN

25. Pseudocyst Percutaneous aspiration: high recurrence rate or persistent pancreaticocutaneous fistula Internal Drainage Cystgastrostomy, Cystenterostomy

26. Gallstone Pancreatitis Common – channel pathogenesis of biliary pancreatitis first proposed by Opie in 1901 Transient blockage of the ampulla of Vater by migratory stone 90% mild attacks (<3 Ranson’s criteria) 10% severe attacks (>3 Ranson’s criteria)

27. Gallstone Pancreatitis A gallstone initiates pancreatitis Early removal of an impacted stone Surgery should be performed during the initial hospitalization after the pancreatitis has subsided. ERCP/sphincterotomy may be of benefit

28. Chronic Pancreatitis Indication for operation Intractable pain Biliary obstruction Duodenal obstruction Suspected carcinoma Preoperative evaluation ETOH/Narcotic Malnutrition or Jaundice Radiographic studies: ERCP/EUS/CT

29. Chronic Pancreatitis Large Duct (>8 mm) Lateral pancreaticojejunostomy (Peustow): 80% effective Small Duct Resective procedures Whipple Total with Auto Islet Transplantation

30. Pancreatic Neoplasms

31. Periampullary Tumors Head of the pancreas (80%) Ampullary (10-15%) Duodenual (4%) Distal Cholangiocarcinoma (3%) Mean age: 62 years Men 65%

32. Periampullary Tumors Pancreatic Carcinoma 20% increase in the last 20 years 4 TH Leading cause of cancer death in women 3 rd Leading cause of cancer death in men Resectability rate 5-15% Regionally unresectable (mesenteric vasculature) Distant metastases Resectability of other periampullary lesions 60-70%

33. Periampullary Tumors Diagnosis Symptoms: weight loss, pain, pruritis Signs: jaundice Laboratory studies: Hyperbilirubinemia, CA 19-9 Pancreas Protocol CT EUS ERCP

34. Periampullary Tumors Surgery Curative procedures Pancreaticoduodenectomy (Whipple) Standard vs. pylorus sparing Local excision Small, localized ampullary lesions in high risk patients

35. Periampullary Tumors Palliative procedures Biliary and/or enteric bypass Five Year Survival Pancreas 0-14% Ampulla 40% Duodenum 30% Distal Bile duct 40% Non-operative therapy Stents: endoscopic, percutaneous Radiation Chemotherapy

36. Cystic Neoplasms Histological Types IPMN – main duct or side branch Serous cystadenoma Mucinous cystadenoma: malignant potential Cystic Neuroendocrine tumor Always rule out pseudocyst

37. Cystic Neoplasms Malignant lesions usually symptomatic Pain, jaundice, mass Survival Serous tumors: excellent Cystadenocarcinoma Resectable : 76% survival Unresectable: mean survival 4 months

38. Neuroendocrine Tumors (islet cell) MEN I: pancreas (80%), pituitary (40%), parathyroid (50%), adrenal cortex (35%) Insulinoma Gastrinoma Glucagonoma VIPoma Somatostatinoma Non-Functioning

39. Neuroendocrine Tumors (islet cell) Insulinoma Most common islet cell tumor 90% benign, solitary Symptoms from neuroglycopenia Diagnosis Whipple’s Triad Fasting blood sugar <50mg.% Symptoms after fasting Relief of symptoms with glucose administration

40. Gastrinoma (Zollinger-Ellison syndrome) First described in 1954 Severe ulcer diathesis Diarrhea caused by a non-beta islet cell tumor of the pancreas. At least 50% are both malignant and multiple.

41. VIPoma (Verner-Morrison, WDHA) Watery diarrhea, hypochloremia, achlorhydria Elevated vasoactive intestinal peptide (VIP) 60% malignant Therapy: surgical excision, streptozotocin, somatostatin/Sandostatin

42. Glucagonoma Extremely rare Clinical syndrome: weight loss, mild diabetes, stomatitis, anemia, migratory necrolytic erythema , diarrhea Elevated serum glucagon 60-70% malignant Treatment: surgical excision, somatostatin/Sandostatin analog

43. Non-functioning Islet Cell Tumors Rare: 1:100,000 population Do not produce detectable hormones Most are solitary lesions Clinical presentation Pain, weight loss Diagnosis: EUS, CT Management Surgery: resection; even metastatic disease Chemotherapy: streptozocin Survival: 58% at 5 years (overall)