Pancreatic cystosis in patients with cystic fibrosis: A qualitative systematic review.

Abstract

BACKGROUND:

Patients with cystic fibrosis (CF) and a CFTR gene mutation may present with a variety of pancreatic disorders. The presence of multiple macrocysts (>1 cm) replacing the entire pancreatic parenchyma is termed pancreatic cystosis. Lack of clear data makes clinical decision making challenging and controversial. The aim of this review is to perform a qualitative systematic analysis of the literature with intention to evaluate management plans.

METHODS:

Electronic databases MEDLINE, Embase, and Scopus were searched for relevant studies, and 19 studies describing patients with pancreatic cystosis were included and analyzed for clinical features and therapy offered.

RESULTS:

The data of 24 patients were collected from included studies. Eight cases (33%) had a documented CFTR gene mutation and 10 (42%) were symptomatic at presentation. Imaging modalities included ultrasound in 18 (75%), CT in 12 (50%), and MRI in 8 (33%) cases. An average size of the largest cyst was 5.4 cm. 6 (25%) patients were offered therapy that described surgical (3), endoscopic (1), or medical therapy (2). Surgeries offered included total pancreatectomy, partial pancreatic resection of uncertain extent, and complex cyst resection. Endoscopic treatment was cystogastrostomy. Novel medical treatment was utilized with Doxepin, Propantheline, and Clonidine, resulting in reduction in cyst size and overall clinical improvement.

CONCLUSION:

Patients with pancreatic cystosis should not be denied treatment when necessary. This literature review is the most comprehensive thus far of cystic fibrosis and pancreatic cystosis, and it did not provide identification of a definitive treatment plan or demonstrate contraindication to specific therapies.

• Authors: Chirag S Desai, Jennifer S Vonderau, Rebecca McCall, Khalid M Khan, Todd H Baron
• Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] (Pancreatology) Vol. 18 Issue 7 Pg. 700-704 (Oct 2018) ISSN: 1424-3911 [Electronic] Switzerland
• PMID: 30139657 (Publication Type: Journal Article, Systematic Review)
• Copyright: Copyright © 2018 IAP and EPC. Published by Elsevier B.V. All rights reserved.

Topics

• Cystic Fibrosis (complications)
• Genetic Predisposition to Disease
• Humans
• Pancreatic Cyst (etiology, pathology, therapy)