Pneumatosis intestinalis (PI) is characterized by multiple gas cysts in the wall of the gastrointestinal tract. Primary PI is extremely rare. In most of the cases PI is due to an underlying disease (traumatic and mechanical, inflammatory and autoimmune diseases, infectious and pulmonary diseases, drug induced, immunosuppression, transplantation, or neoplasm). A 69-year-old woman was treated with mixed connective tissue disease and PI twice operatively and once conservatively in our department. Review of the english literature showed 13 more cases of PI with underlying mixed connective tissue disease.
Most published cases of pneumatosis intestinalis with radiological finding of pneumoperitoneum were treated conservatively and should have not been considered as a reason for surgery. Therefore, the treatment of PI can present as a major dilemma for the surgeon.
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