Distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
Diagnosis almost certain
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Cystic fibrosis
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Young adult with established cystic fibrosis, presents with small bowel obstruction due to impaction of thick mucus.
The appendix is distended with hyperdense material.
Note the complete fatty atrophy of the pancreas.
Case Discussion
Young adult with established cystic fibrosis presents with small bowel obstruction due to impaction of thick mucus.
The appendix is distended with hyperdense material
- the appendix is routinely distended (>6mm) in the absence of appendicitis resulting from mucoid impaction and the secondary signs to diagnose appendicitis is critical
- despite the common distention of the appendix by inspissated secretions, the reported prevalence of acute appendicitis in CF patients is lower than that in the general population
Note the complete fatty atrophy of the pancreas.
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