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Updated: Jun 27, 2025
Rectal Organoid Morphology Analysis ROMA: A Diagnostic Assay in Cystic Fibrosis
Published on: June 10, 2022
Presentation, characteristics and management of obstructive intestinal conditions in cystic fibrosis
Caitlin Miles1, Natalie Ling2, Eldho Paul3
1Nutrition and Dietetics, Monash Children's Hospital, Melbourne, Victoria, Australia.
Insights
Cystic fibrosis (CF) patients are more often hospitalized for constipation than distal intestinal obstruction syndrome (DIOS). Current diagnostic and treatment practices vary, indicating a need for better adherence to established guidelines for CF gastrointestinal care.
Area of Science:
- Gastroenterology
- Pulmonology
- Pediatrics
- Internal Medicine
Background:
- Constipation and distal intestinal obstruction syndrome (DIOS) are frequent gastrointestinal complications in cystic fibrosis (CF).
- Effective management of these conditions is crucial for improving the quality of life in CF patients.
- Understanding current hospitalization patterns and diagnostic adherence is essential for optimizing care.
Purpose of the Study:
- To characterize hospitalizations for constipation and DIOS in a pediatric and adult CF population over a 12-year period.
- To assess the proportion of these events meeting European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) criteria.
- To describe the management strategies employed for constipation and DIOS in CF patients.
Main Methods:
- A retrospective analysis of CF patients hospitalized with constipation or DIOS between 2011 and 2022.
- Retrospective application of ESPGHAN definitions to determine diagnostic accuracy for constipation and DIOS admissions.
- Review of treatment protocols, including medication use and enema administration.
Main Results:
- 42 hospitalizations for constipation and 33 for DIOS were recorded.
- 88.10% of constipation admissions met ESPGHAN criteria, versus only 3.0% for DIOS.
- Polyethylene glycol (PEG) was the primary treatment; enemas were more common in DIOS, and weaning PEG doses were less likely in DIOS patients.
Conclusions:
- Hospital admissions for constipation are more frequent than for DIOS in CF patients.
- Significant variability exists in diagnostic and therapeutic practices for constipation and DIOS.
- There is a clear need to improve the implementation and adoption of existing best-practice guidelines for CF gastrointestinal management.
Objective:
Constipation and distal intestinal obstruction syndrome (DIOS) are common gastrointestinal manifestations of cystic fibrosis (CF). The primary aim was to describe the characteristics of constipation and DIOS hospitalisations in a paediatric and adult CF service over a 12-year period. The secondary aims were to determine the proportion of constipation and DIOS presentations which met the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) CF Working Group definitions and to describe management strategies of both conditions.
Method:
A retrospective study of children and adults with CF who were admitted with a primary diagnosis of constipation or DIOS between 1 January 2011 and 31 December 2022. ESPGHAN definitions for constipation and DIOS were retrospectively applied to all admissions to determine if the primary medical diagnosis met ESPGHAN criteria.
Results:
During the 12-year study period, 42 hospitalisations for constipation were recorded in 19 patients, and 33 hospitalisations for DIOS were recorded in 23 patients. 88.10% of constipation episodes met ESPGHAN definitions, compared with 3.0% of DIOS episodes. Constipation and DIOS were primarily treated with polyethylene glycol (PEG). The use of sodium amidotrizoate meglumine enemas was significantly higher in the DIOS group (p=0.045). Those admitted with DIOS were significantly less likely to be recommended a weaning dose of PEG (p=0.018).
Conclusion:
Children and adults with CF are more commonly admitted for the management of constipation than DIOS. There is considerable variation in diagnostic and therapeutic practice, and this study highlights the need to enhance the translation and adoption of existing best-practice guidelines.
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