Primary pneumatosis intestinalis (PPI) is a benign idiopathic condition in which multiple gas-filled cystic lesions are seen in the gastrointestinal tract wall. The changes are usually seen initially on radiography or CT with CT being the more sensitive test.
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Epidemiology
Primary pneumatosis intestinalis is rare and was shown in one study to comprise 15% of cases of all intestinal pneumatoses 3.
Clinical presentation
This condition is usually asymptomatic and presents as an incidental finding on medical imaging.
Pathology
Multiple gas-filled cysts are seen in the mucosa and submucosa of the small and large bowel.
Differential diagnosis
- ischaemic bowel: gas in the ischaemic bowel wall has a linear or bubble-like pattern, in contrast to the PPI gas pattern, which is circular
- causes of secondary pneumatosis intestinalis
- post-instrumentation of the bowel e.g. endoscopy, surgery
- COPD, ventilated patients
- drugs such as steroids, chemotherapy (e.g. bevacizumab)
- pseudopneumatosis: intraluminal gas trapped between faeces and the bowel wall