1. Chronic Pancreatitis in Children
Shannon Cannon
December 5, 2013
Presentation and Surgical Management

2. Case Presentation
A.D. is an 11 yo F with diagnosis of chronic pancreatitis, referred from GI Clinic. She first presented with pancreatitis in and has had multiple subsequent episodes of progressively worsening length/severity. Her symptoms have worsened in last 3 months. Her main presenting symptom is abdominal pain.
PMH: Choledocholithiasis
PSH:
Appendectomy 2009
Cholecystectomy 8/2012
ERCP with sphincterotomy x3, most recently on 9/2013

3. Case Presentation (continued)
Chronic abdominal pain
Taking amitriptyline, oxycodone, and ibuprofen regularly
Seen in pain medicine clinic for chronic pain associated with pancreatitis
SPINK1 positive

4. ERCP

5. Pediatric Pancreatitis
Classifications: Acute Pancreatitis, Acute Recurrent Pancreatitis, Chronic Pancreatitis
Subset of chronic – genetic/hereditary pancreatitis
Acute – similar presentation to adult
Epigastric pain radiating to back, N/V
Chronic – irreversible inflammatory process with histologic changes in pancreatic tissue and subsequent dysfunction

6. Definitions
INSPPIRE consortium (International Study Group of Pediatric Pancreatitis: In Search of a Cure)
J Pediatr Gastroenterol Nutr 2012;55: 261 – 265

7. Epidemiology Incidence of all pancreatitis in children: 1/50,000
Chronic pancreatitis is rare hereditary pancreatitis more rare
Causes of chronic pancreatitis in Children
Higher percentage idiopathic (30-50%) compared to adult population
Obstructive: pancreas divisum, choledochal cysts, duplication of pancreatic duct, trauma, idiopathic fibrosing pancreatitis

8. Microscopic findings: Inflammatory cell infiltrate (predominantly chronic), fibrosis, loss of acinar tissue, relative sparing of the islets of Langerhans
Gastrointestinal and Liver Pathology, Ch.17,

9. Presentation Abdominal Pain Less common presentations:
Neuropathy, Interstitial and ductal hypertension, Chronic inflammation, or Pseudocyst
Less common presentations:
Duodenal/colonic obstruction
Splenic vein occlusion
Suspicion of a malignant neoplasm

10. Diagnosis Labs Imaging: “chain of lakes,” dilated pancreatic duct
Exocrine: fecal elastase, amylase, lipase
Endocrine: blood glucose
Genetic mutations
Imaging: “chain of lakes,” dilated pancreatic duct CT
ERCP
MRCP
Semin Pediatr Surg Aug;21(3):

11. Hereditary Factors Associated gene mutations
CFTR, PRSS1, SPINK1
Rare…but common among those with chronic pancreatitis
79% of patients in a single-site retrospective study
Increased risk of pancreatic adenocarcinoma
J Pediatr Gastroenterol Nutr May;54(5):645-50

12. Puestow Procedure (Lateral Pancreaticojejunostomy)
Drainage procedure (as opposed to resection procedure)
Major steps
Transverse supraumbilical or a Chevron incision.
Duodenum mobilization, exposure of the anterior pancreas
Identification of the dilated pancreatic duct, on palpation or ultrasound, confirmed with needle aspiration.
Incise dilated pancreatic duct, removing stones if present
35 cm Roux-en-Y jejunal limb creation, passing roux limb retrocolic to the pancreas

14. J Gastrointest Surg 2009 May;13(5):1004-8

15. Semin Pediatr Surg. 2012 Aug;21(3):266-71.

16. Patient Selection Refractory abdominal pain
Attempts at treatment with ERCP & pancreatic duct stent placement have failed

17. 32% went on to surgical intervention, 12.7% with Puestow Conclusions
Retrospectively selected 87 patients with hereditary pancreatitis ( )
52% women, median age 16.9±1.7
68% confirmed hereditary with genetic testing (16% SPINK1)
32% went on to surgical intervention, 12.7% with Puestow
Conclusions
21% of patients treated endoscopically first required a subsequent surgery for recurrent symptoms, 64 % of patients treated with surgery required a subsequent endoscopic procedure (p < 0.01).
Time between interventions: 2-3 years with endoscopy, years with surgery

18. 7 patients with follow-up, 5 completely pain-free
Retrospective study of 32 pediatric patients with chronic pancreatitis ( )
15 patients with surgical intervention, 9 with Puestow procedure (47%, 28%)
Mean age at time of surgery 6.0±4.1 years
Mean time presentation to operation 3.3±3.3 years
7 patients with follow-up, 5 completely pain-free

19. 5 patients previously failed ERCP treatment
Retrospective series of 6 patients with chronic pancreatitis with Puestow procedure
3/6 patients had SPINK1 mutation
Median age 7.5 years
Median time presentation to operation 4 years
5 patients previously failed ERCP treatment
Improved pain in all patients but variable long-term results
Recurrence of pain, eventual pancreatectomy (n=2, both hereditary)
Recurrence of acute pancreatitis episodes, overall improved pain (n=2, 1 hereditary)
Pain free, too soon for follow-up (n=2)
Conclusion: Safe to perform, variable pain outcomes with hereditary pancreatitis

20. Case Recap
11 yo F with hereditary, chronic pancreatitis presenting with severe pain, refractory to endoscopic interventions. SPINK1 mutation positive.
Implication of SPINK1 mutation in outcome for this patient?
Need for further interventions in the future?

21. Conclusions
Chronic pancreatitis is rare in children (hereditary pancreatitis even rarer)
Puestow procedure is a drainage procedure used to treat refractory cases of chronic pancreatitis
Low-powered studies are not strongly conclusive in demonstrating lasting outcomes or long-term efficacy of Puestow.

22. Future Directions Robotic Puestow
Meehan J & Sawin RR, Robotic lateral pancreaticojejunostomy (Puestow). J Pediatr Surg Jun;46(6):e5-8
Eid GM et al. Robotic-assisted laparoscopic side-to-side lateral pancreaticojejunostomy. J Gastrointest Surg Jul;15(7):1243
Further identifying genetic causes of chronic pancreatitis
Masson E et al. A conservative assessment of the major genetic causes of idiopathic chronic pancreatitis: data from a comprehensive analysis of PRSS1, SPINK1, CTRC and CFTR genes in 253 young French patients. PLoS One Aug 8;8(8):e73522

23. Questions?

24. References
Morinville VD, et al. Definitions of Pediatric Pancreatitis and Survey of Present Clinical Practices. J Pediatr Gastroenterol Nutr ;55:261 – 265.
Dzakovic A & Superina R. Acute and chronic pancreatitis: surgical management. Semin Pediatr Surg Aug;21(3):
Ceppa EP, et al. Hereditary pancreatitis: endoscopic and surgical management. J Gastrointest. Surg May;17(5):847-56; discussion 856-7
Sultan M, et al. Genetic prevalence and characteristics in children with recurrent pancreatitis. J Pediatr Gastroenterol Nutr May;54(5):645-50
Ceppa EP and Pappas TN. Modified puestow lateral pancreaticojejunostomy. J Gastrointest Surg May;13(5):1004-8
Clifton MS, et al. Surgical treatment of childhood recurrent pancreatitis. J Pediatr Surg Jul;42(7):
Miqdady M and Kitagawa S. Causes of chronic pancreatitis in children and adolescents. Up To Date, accessed 12/4/2013
DuBay D, et al. The modified Puestow procedure for complicated hereditary pancreatitis in children. J Pediatr Surg Feb;35(2):343-8
Iacobuzio-Donahue CA & Montgomery E. (2012) Gastrointestinal and Liver Pathology. “Non-neoplastic and neoplastic pathology of the pancreas” 2nd Ed. Ch. 17: