Outcomes of Interventional ERCP in Hereditary Pancreatitis

Abstract

Purpose: Hereditary Pancreatitis (HP) is an autosomal dominant disease characterized by recurrent acute pancreatitis progressing to chronic pancreatitis. In addition to supportive measures, traditional treatment has included surgical decompression or resection. There are limited data evaluating the outcomes and role of interventional ERCP in HP. The aims of this study were to evaluate patients with HP before the first and after the first and last interventional ERCP (s). Methods: From 1990 to 2008, 21 patients with HP received care at our institution and were retrospectively assessed for response to therapeutic ERCP. Medical records were reviewed and a telephone survey was conducted to obtain information. Data collected included documentation of hereditary etiology, ERCP interventions and complications, pancreatic surgeries, pre and post ERCP pain levels, daily oxycodone equivalent usage, yearly hospitalizations, incidence of diabetes, and pancreatic enzyme usage. Statistical methods included a Wilcoxon Signed Rank test and a box plot background. A box plot gives a compact display of the distribution of a variable. Each measure of a change has been calculated as the pre ERCP value minus a post ERCP value; Differences greater than zero indicate a benefit from the procedure. Results: 21 patients underwent a total of 87 interventional ERCPs (mean 4, range 1–9) and were followed up for a mean period of 5 years (2–212 months). Interventions included sphincterotomy, stone extraction, duct dilation, and stent placement. The mean patient age at diagnosis, first ERCP, and follow up was 15, 19 (2–39), and 28 years, respectively. 11 of 12 patients (92%) who received surgical therapy required subsequent interventional ERCPs. Pre and post first ERCP mean pain scores decreased from 8.3 to 3.2 (P= .001) and post last ERCP scores decreased to 2.7 (P= .001). Yearly hospitalizations decreased from 5.7 to 1.9 (P < .001) and then to 1.6 (P= .001). Daily oxycodone equivalent usage decreased from 39 mg to 34 mg (P= .7) and then to 9.4 mg (P < .05). Complications included pancreatitis in 3% with no perforations, bleeding, or infection. Conclusion: 1) Despite decompressive or resective surgeries in 12 of 21 patients, the majority (92%) developed subsequent complications and required endoscopic therapy. 2) Endoscopic management for amenable lesions often requires multiple ERCPs. 3) Interventional ERCPs in patients with HP is associated with decreased pain, analgesic usage, hospitalizations, and episodes of recurrent pancreatitis.Table: Surgical Therapy.