Presentation, characteristics and management of obstructive intestinal conditions in cystic fibrosis.

Abstract

Objectives To determine the number of constipation and DIOS episodes requiring hospitalisation in a combined paediatric and adult CF service over a 10 year period. Secondary aims include the exploration of diagnostic and management practices. Methods Cross sectional retrospective (2011-2020) exploration of children and adults with CF who were admitted with a primary diagnosis of constipation or DIOS to Monash Health (Australia). Results 33 hospitalisations for constipation were recorded in 21 patients; 63.6% (21 of 33) in children. 28 hospitalisations for DIOS were recorded in 21 patients; 50.0% (14 of 28) in children. 87.9% (29 of 33) of constipation episodes met ESPGHAN CF Working Group definitions when applied retrospectively against the primary medical diagnosis; no DIOS episodes met ESPGHAN definitions. Laxative use pre-admission was significantly higher in the constipation group compared with the DIOS group; 69.7% vs. 25.5% (p = 0.0003). 3 of 28 (11%) DIOS episodes required surgery. No patients with constipation required surgical intervention. Symptomatic treatment with IV hydration, fasting and opioids was more commonly used in DIOS than in constipation. The use of PEG laxatives in the acute management of both DIOS and constipation was high; 75.5% (21 of 28) and 84.8% (28 of 33). Treatment with oral diatrizoate meglumine/diatrizoate sodium was more than twice as likely in DIOS than in constipation (32.1% vs. 12.1%; p = 0.190). PEG laxatives were significantly more likely to be prescribed as a post-discharge treatment in constipation than in DIOS, 81.8% vs. 42.9% (p = 0.005). Conclusion In a combined paediatric and adult CF centre in Australia, hospitalisation for the management of constipation is as common as that of DIOS. Disparity in the diagnostic and therapeutic care provided is evident and this warrants the tailored implementation of a site-specific clinical guideline on evidence-based diagnostic and therapeutic pathways for constipation and DIOS in CF.Copyright © 2023 European Cystic Fibrosis Society