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Functional Pancreatic Sphincter Dysfunction in Children

Recommendations for Diagnosis and Management

Lin, Tom K.*; Fishman, Douglas S.; Giefer, Matthew J.; Liu, Quin Y.§; Troendle, David||; Werlin, Steven; Lowe, Mark E.#; Uc, Aliye**

Journal of Pediatric Gastroenterology and Nutrition: December 2019 - Volume 69 - Issue 6 - p 704–709
doi: 10.1097/MPG.0000000000002515
Original Article: Pancreatology

Objective: Functional pancreatic sphincter dysfunction (FPSD), previously characterized as pancreatic sphincter of Oddi dysfunction, is a rarely described cause of pancreatitis. Most studies are reported in adults with alcohol or smoking as confounders, which are uncommon risk factors in children. There are no tests to reliably diagnose FPSD in pediatrics and it is unclear to what degree this disorder contributes to childhood pancreatitis.

Methods: We conducted a literature review of the diagnostic and treatment approaches for FPSD, including unique challenges applicable to pediatrics. We identified best practices in the management of children with suspected FPSD and formed a consensus expert opinion.

Results: In children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP), we recommend that other risk factors, specifically obstructive factors, be ruled out before considering FPSD as the underlying etiology. In children with ARP/CP, FPSD may be the etiology behind a persistently dilated pancreatic duct in the absence of an alternative obstructive process. Endoscopic retrograde cholangiopancreatography with sphincterotomy should be considered in a select group of children with ARP/CP when FPSD is highly suspected and other etiologies have been effectively ruled out. The family and patient should be thoroughly counseled regarding the risks and advantages of endoscopic intervention. Endoscopic retrograde cholangiopancreatography for suspected FPSD should be considered with caution in children with ARP/CP when pancreatic ductal dilatation is absent.

Conclusions: Our consensus expert guidelines provide a uniform approach to the diagnosis and treatment of pediatric FPSD. Further research is necessary to determine the full contribution of FPSD to pediatric pancreatitis.

*Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Baylor College of Medicine; Texas Children's Hospital, Houston, TX

Seattle Children's Hospital, University of Washington, Seattle, WA

§Cedars-Sinai Medical Center, Los Angeles, CA

||University of Texas Southwestern Medical Center, Dallas, TX

Medical College of Wisconsin, Milwaukee, WI

#Washington University School of Medicine, St. Louis, MO

**Stead Family Children's Hospital, University of Iowa, Iowa City, IA.

Address correspondence and reprint requests to Aliye Uc, MD, Stead Family Department of Pediatrics, The University of Iowa Carver College of Medicine, BT1120-C, 200 Hawkins Dr, Iowa City, IA 52242 (e-mail:

Received 16 December, 2018

Accepted 11 September, 2019

This work was supported by NIH U01 DK108334, R01 DK118752, and R24 DK096518 (A.U.). The sponsors of this study (NIH) did not participate in the study design, collection of data, writing of the report, or the decision to submit this manuscript for publication.

A.U. is a member of Pediatric Gastroenterology Sub-board at American Board of Pediatrics.

The remaining authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,