1. Chronic Pancreatitis in Children:
Focus on Pancreatic Divisum & Genetic Pancreatitis
Case Conference
Joanna Yeh
October 27, 2011

2. Case Presentation
• 7 yo girl admit to CHLA in April 2011 for acute
onset emesis and pain
• Pain was periumbilical, causing her to be sent
home from school
• Pain seems to be worse with food and liquids
• She had low grade temperatures x 1 day at
presentation

3. • Patient has had abdominal pain episodes for 2
years; diagnosed with “constipation”
• Prior to admission, patient had been seen by
PMD and told she had “stomach flu”
• When family history was elicited, dad has
hereditary pancreatitis (PRSSI and CFTR)
• Remaining H&P was non contributory
Further History

4. Physical Exam
• Temp 100.1 HR 121 BP 102/58 RR 20 Sat 99% RA
• Wt. 20 kg
• Abd: soft, non tender to deep palpation
throughout but endorses intermittent subjective
pain. Not distended, no masses. No HSM.
• Skin: no rashes, no papules
• Remaining exam normal

5. • At CHLA, labs notable for normal CBC, normal
CMP, lipase 1030
• Abdominal ultrasound notable for prominent
pancreatic duct, 3-4 mm, common bile duct 1
mm
• Abdominal CT was done with IV and PO
contrast that showed no pancreatic
inflammation
• MR cholangiopancreatography (MRCP) revealed
pancreas divisum
Labs & Imaging

7. Chronic Pancreatitis Definition
• Progressive inflammatory disease of pancreas
• Characterized by irreversible structural
changes that result in irreversible exocrine and
endocrine pancreatic insufficiency

8. Etiology
• Congenital anomalies
• Genetic
• Toxic/metabolic
• Autoimmune
• Idiopathic

9. Congenital anomalies
• Pancreatic divisum
• Choledochal cysts
• Pancreatic duct duplication
• Renal cysts
• Congenital pancreatic cysts
• Annular pancreas

10. Embryology

11. Anatomy

15. Different types of pancreas divisum

16. Divisum
• Diagnosis:
– Endoscopic ultrasonography
– ERCP (endoscopic retrograde cholangiopancreatography)
– MRCP (magnetic resonance cholangiopancreatography)
• Divisum can be the sole etiology of acute or
chronic pancreatitis, but not always
• Incidental divisum: 5-10% lifetime risk of
developing pancreatitis
• Malignancy risk highest in dorsal pancreas

18. Divisum Treatment
• Endoscopy / Therapeutic ERCP
– Spincterotomy
– Papillary dilation
– Stent insertion
• Surgical
– Minor papilla spincterotomy
– Puestow procedure (pancreaticojejunostomy)
– Pancreatectomy

19. Genetic Pancreatitis
• PRSS1
cationic trypsinogen
• SPINK1
serine protease inhibitor Kazal type 1
• CFTR
cystic fibrosis transmembrane conductance regulator
• Chymotrypsin C

22. Who should be tested?
– A family history of recurrent acute pancreatitis,
idiopathic chronic pancreatitis, or childhood
pancreatitis without a known cause
– Relatives known to carry mutations associated with
hereditary pancreatitis
– Recurrent acute attacks of pancreatitis for which there
is no identifiable cause
– An unexplained documented episode of pancreatitis
as a child
– Idiopathic chronic pancreatitis, particularly when the
onset of pancreatitis occurs before age 25

24. Genetic Pancreatitis: Management
• Small meals, low fat, good hydration
• Avoidance of alcohol and tobacco
• Monitor exocrine and endocrine function
– Enzyme supplementation
– Diabetes management
• Pancreaticojejunostomy / Pancreatectomy
• Islet cell autotransplantation

25. Pancreatic Enzymes
• Child<4 yo: 1000 units lipase/kg/meal
• Child>4 yo: 500 units lipase/kg/meal
• Max: 2500 units lipase/kg/meal
• Snack dose is about ½ of meal dose
• Enteric coated microspheres
• At UCLA inpatient, we have Zenpep (5) and Zenpep (20)
• Others: Creon, Pancreacarb, Pancreaze
• If sprinkle into food, should be relatively acidic to avoid
dissolving enteric coating (applesauce, banana, sweet
potato)

26. Patient Update
• Hospitalized in Oct 2011 to Santa Monica for
another episode of pancreatitis
• Father has since undergone pancreatectomy
and islet cell transplantation
• Her genetic testing was positive for CFTR (dad
is positive for CFTR and PRSSI)
• Dr. Farrell is following her and may consider
EUS in future. No plans for ERCP.

27. References
• Takuma, et al, “Pancreatic disease associated with pancreas divisum,” Digestive Surgery, 2010, 27,
pg 144-8.
• Dumont, et al, “Pancreas divisum and the dominant dorsal duct syndrome,” Annals of Surgery, Jan
2005, 130, 1, pg 5-14.
• Bellin, et al, ‘Outcome After Pancreatectomy and Islet Autotransplantation in a Pediatric
Population,” Journal of Pediactirc Gastroenterology and Nutrition, 2008, 47, p 37-44.
• “Surgical Treatment of Pancreas Divisum Causing Chronic Pancreatitis,” Journal of Gastrointestinal
Surgery, May 2005, 9, 5, pg 710-5.
• Dray, et al, “Association of Pancreas Divisum and Recurrent Acute Pancreatitis,” Pancreas, 2007,
35, pg 90-3.
• Bhardwaj, et al, “A RCT of antioxidant supplementation for pain relief in patients with chronic
pancreatitis,” Gastroenterology, 2009, 136, 1, pg 149.
• Kamisawa, et al, “Pancreatitis associated with congenital abnormalities of the pancreaticobiliary
system,” Hepatogastroenterology, 2005, 52, 61, pg 223-9.
• Rosendahl, et al, “Hereditary chronic pancreatitis,” Orphanet Journal of Rare Diseases,” Jan 2007,
2, 1.
• Witt H, “Genetics of pancreatitis: a guide for clinicians,” Digestive Diseases, 2010, 28, 6, pg 702-8.
• Ferrone, et al, “Panreatic enzyme pharmacotherapy,” Pharmacotherapy, 2007, 27, 6, pg 910-20.