Pancreas Divisum: Patient Presentation and Discussion

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September 2001 Pancreas Divisum: Patient Presentation and Discussion Hilary Hochberg Advanced Radiology Clerkship Beth Israel Deaconess Medical Center Dr. Gillian Lieberman

Patient JC 44 yo male Abdominal pain, epigastric, radiating to back x 1d Nausea, vomiting, diaphoresis, low-grade fevers, chills PMHx: 4 m ago, EU abdominal pain Normal CT 2

Physical Exam Vitals: T 100.3 P70 RR20 BP 150/90 Abd: Distended, tenderness to palpation diffusely, mostly periumbilical -Rebound +Voluntary guarding BS markedly depressed No Cullen/Grey Turner sign 3

Labs CBC: WBC 13.4 Hct 42 Plets 257 Amylase 2302 Lipase 1940 LFTs: ALT/AST 47/23 LDH 199 Alk Phos 83 TG 264 E lytes: 143 104 23 / 4.3 25 0.9 \ 150 Ca 9.3 / Ph 4.1/ Mg 2.0 4

Our Patient JC: Abdomenal CT Fat stranding (misty appearance) of peripancreatic fat Indistinctive pancreatic margins Thickening of pararenal fascia and pararenal fluid accumulation Findings consistent with pancreatitis 5

Anatomy of the Pancreas Illustration at http://www.hopkins-gi.org/subspecialties/chronic/introduction/anatomy.htm 6

JC:Endoscopic Retrograde What a mouthful! Cholangiopancreatography (ERCP) Cannulation of patient JC s major ampulla Cystic duct (corkscrew) and gall bladder CBD Illustration at http://www.aafp.org/afp/990501ap/2507.html 7

Our Patient JC: ERCP Normal intrahepatic ductal system 8

JC: ERCP Major papilla cannulation Minor papilla cannulation Small ventral duct Dominant dorsal duct connecting to main pancreatic duct 9

Ductal Variations Minor Major Minor Major Minor Major 10

Ductal Variation Type 3 Pancreas Divisum The most common congenital variant of pancreatic anatomy. 11

Magnetic Resonance Cholangiopancreatography Stomach Fluid filled bile and pancreatic ducts are very bright on T2 images Gall Bladder Accessory duct drains majority of pancreas Pancreas Divisum Anomaly 12

stomach Our Patient JC: 2 Weeks later J.C. clinically improved, and he was discharged home. 2 weeks later: He returned to EU with nausea, vomiting, and periumbilical pain, similar to prior admission. CT: Pancreatitis and large pancreatic pseudocyst extending into lesser sac and compressing stomach 13

So What happened?

Pancreas Divisum: Embryology Picture: http://anatomy.med.unsw.edu.au/cbl/embryo/sysnote.htm 15

Normal Embryological Development of Pancreas Week 4: Dorsal pancreatic bud Week 5: Ventral bud appears between GB and duodenum. Bile duct moves to right, apposing the pancreatic buds as duodenal wall differentially grows. Illustrations: www.vesalius.com Dorsal duct (Santorini) and ventral duct (Wirsung) FUSE. The ventral duct is the main duct for pancreatic secretions into the duodenum. If the dorsal duct persists, it is called the 16 minor papilla.

Pancreas Divisum NO FUSION of ventral and dorsal pancreatic buds Ventral bud only drains ventral pancreas Dorsal bud (through minor papilla) must drain majority of pancreas. Minor papilla is often stenotic and inhibits flow of pancreatic juice Pancreatitis Other features: Stenosis of minor papilla Signs of chronic pancreatitis Dilated dorsal duct Santorinicele 17

Diagnosis of Pancreas Divisum: ERCP Absent or small ventral duct Confirm by cannulation of minor duct lack of communication between dorsal and ventral and dorsal ducts. 18

Complications of Pancreatitis Acute Loculated fluid collection Pancreatic necrosis Pancreatic hemorrhage Subacute Phlegmon Pseudocyst Pancreatic ascites Abscess Pseudoaneurysm 19

Treatment of Pancreas Divisum Standard Medical Therapy: Low fat diet Analgesics Pancreatic enzymes Anticholinergics Minor Papilla Treatment: Endoscopic Dilatation Stenting Papillotomy Open surgical Minor sphincteroplasty Pancreatico-jejunostomy with Roux-en-Y limb 20

Pros Cons ERCP MRCP Best visualization of ductal anatomy Access for biopsy or therapeutic intervention (sphincterotomy) Noninvasive Better imaging of parenchyma Technically difficult. Radiation Expensive Invasive: Complications (4% ERCP pancreatitis) Worse resolution than fluoroscopy so less sensitive than ERCP Screening Examination In Patients With Low or MRCP growing in use. Intermediate Probability Of choledocholithiasis Failed or Incomplete ERCP Post-operative Anatomy Primary Sclerosing Cholangitis (PSC) Complications of Chronic Pancreatitis Variant Ductal Anatomy! 21

Articles of interest: Ertan A. Long-term results after endoscopic pancreatic stent placement without pancreatic papillotomy in acute recurrent pancreatitis due to pancreas divisum. Gastrointestinal Endoscopy 2000; 52(1): 9-12. Freeman M, et al. Risk factors for post-ercp pancreatitis: A prospective, multicenter study. Gastrointestinal Endoscopy 2001; 54(4) 425-434. Varshney S, Johnson CD. Pancreas Divisum. International Journal of Pancreatology 1999; 25 (2): 135-141. 22

Acknowledgments Pamela Lepkowski Larry Barbaras & Cara Lyn D amour The End 23