General
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• Submucosal gas-filled cysts within gastrointestinal tract

Terminology
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• Called mucosal pseudolipomatosis if it resembles lipomatosis microscopically (Am J Clin Pathol 1985;84:575)

Sites
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• Entire gastrointestinal tract can be affected

Etiology
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• In infants, fulminant form is associated with necrotizing enterocolitis and may be fatal
• Also associated with cystic fibrosis or congenital heart defects
• In adults, either idiopathic or associated with obstruction, chronic lung disease, scleroderma, chemotherapy, drugs, ischemic colitis, vascular insufficiency, or infection with gas-producing bacteria, such as Clostridia (Am J Med Sci 2006;332:100)

Clinical features
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• Either benign or fulminant form
• Often indolent clinical course, although radiographically can resemble carcinoma
• Patients may have nausea, vomiting, diarrhea
• Considered a finding, not a diagnosis

Radiology
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• Multiple, thin-walled, air-filled submucosal or subserosal cysts
• Air may be present in peritoneum

Case reports
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• 6 year old girl with ulcerative colitis (Arch Pathol Lab Med 1999;123:354)
• 25 year old woman with abdominal pain for 10 years and pneumoperitoneum (N Engl J Med 2009;361:e12)

Treatment
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• Surgery if bowel necrosis, perforation, or peritonitis

Clinical images
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Coronal computed tomography

Gross description
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• Polypoid grapelike masses protruding through mucosa

Gross images
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Multiple subserosal bubbles

Diffuse hemorrhage

 

Pneumatosis intestinalis

Pneumatosis intestinalis and focal mucosal necrosis

Micro description
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• Empty submucosal cysts lined by multinucleated giant cells
• Mucosa may demonstrate cryptitis, crypt abscesses, granulomas
• Can also resemble lipomatosis

Micro images
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Submucosal gas-filled cysts

Multinucleated giant cells

6 year old girl with ulcerative colitis

Videos
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• Colon – Pneumatosis Cystoides Intestinalis

Differential diagnosis
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• Crohn disease

Additional references
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• eMedicine-Pneumatosis Intestinalis Imaging

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