Journal of Gastrointestinal Surgery

, Volume 17, Issue 5, pp 847–857

Hereditary Pancreatitis: Endoscopic and Surgical Management

  • Eugene P. Ceppa
  • Henry A. Pitt
  • JoAnna L. Hunter
  • Charles M. Leys
  • Nicholas J. Zyromski
  • Frederick J. Rescorla
  • Kumar Sandrasegaran
  • Evan L. Fogel
  • Lee W. McHenry
  • James L. Watkins
  • Stuart Sherman
  • Glen A. Lehman
2012 SSAT Plenary Presentation

DOI: 10.1007/s11605-013-2167-8

Cite this article as:
Ceppa, E.P., Pitt, H.A., Hunter, J.L. et al. J Gastrointest Surg (2013) 17: 847. doi:10.1007/s11605-013-2167-8

Abstract

Introduction

Hereditary pancreatitis is a rare cause of chronic pancreatitis. In recent years, genetic mutations have been characterized. The rarity of this disorder has resulted in a gap in clinical knowledge. The aims were to characterize patients with hereditary pancreatitis and establish clinical guidelines.

Methods

Pediatric and adult endoscopic, surgical, radiologic, and genetic databases from 1998 to 2012 were searched. Patients with recurrent acute or chronic pancreatitis and genetic mutation for either PRSS-1, SPINK-1, or CFTR or those who met the family history criteria were included. Patients with pancreatitis due to other causes, without a positive family history, familial pancreatic cancer, or cystic fibrosis, were excluded.

Results

Eighty-seven patients were identified. Genetic testing confirmed the diagnosis in 54 patients (62 %). Eighty-five patients (98 %) underwent 263 endoscopic procedures including sphincterotomy (72 %), stone removal (49 %), and pancreatic duct stenting (82 %). Twenty-eight patients (32 %) have undergone 37 operations which included 19 resections and 18 drainage procedures. The interval between procedures for recurrent pain was longer for surgery than for endoscopic therapy (9.1 vs. 3.4 years, p < 0.05).

Conclusions

Most children and young adults with hereditary pancreatitis can be managed initially with endoscopic therapy. When surgery is undertaken, the procedure should be tailored to the pancreatic anatomy and cancer risk.

Keywords

Hereditary pancreatitis Acute pancreatitis Chronic pancreatitis PRSS1 SPINK1 CFTR Endotherapy Endoscopic retrograde cholangiopancreatography Endoscopic ultrasound surgery 

Abbreviations

ERCP

Endoscopic retrograde cholangiopancreatography

EUS

Endoscopic ultrasound

CT

Computed tomography

MRI

Magnetic resonance imaging

Copyright information

© The Society for Surgery of the Alimentary Tract 2013

Authors and Affiliations

  • Eugene P. Ceppa
    • 1
  • Henry A. Pitt
    • 1
  • JoAnna L. Hunter
    • 1
  • Charles M. Leys
    • 2
  • Nicholas J. Zyromski
    • 1
  • Frederick J. Rescorla
    • 2
  • Kumar Sandrasegaran
    • 3
  • Evan L. Fogel
    • 4
  • Lee W. McHenry
    • 4
  • James L. Watkins
    • 4
  • Stuart Sherman
    • 4
  • Glen A. Lehman
    • 4
  1. 1.Department of SurgeryIndiana University Medical CenterIndianapolisUSA
  2. 2.Section of Pediatric SurgeryIndiana University Medical CenterIndianapolisUSA
  3. 3.Department of RadiologyIndiana University Medical CenterIndianapolisUSA
  4. 4.Division of GastroenterologyIndiana University Medical CenterIndianapolisUSA

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