Pneumatosis cystoides intestinalis

Colon (tumor & nontumor)

Other nonneoplastic

Topic Completed: 1 October 2013

Revised: 9 January 2019, last major update October 2013

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Cite this page: Gonzalez R. Pneumatosis cystoides intestinalis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/colonpneumatosis.html. Accessed August 15th, 2020.

Definition / general

Submucosal gas filled cysts within gastrointestinal tract

Terminology

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

Sites

Entire gastrointestinal tract can be affected

Etiology

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

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 description

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

Case reports

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