Colon
Other nonneoplastic
Pneumatosis cystoides intestinalis


Topic Completed: 4 February 2021

Minor changes: 4 February 2021

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PubMed Search: pneumatosis cystoides intestinalis colon [title]

Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Pneumatosis cystoides intestinalis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/colonpneumatosis.html. Accessed June 19th, 2021.
Definition / general
  • Submucosal gas filled cysts within gastrointestinal tract
Essential features
  • Gas filled spaces throughout the colon, often lined with giant cells
  • May occur in children and adults and be benign or fulminant
  • May require surgery
Terminology
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
Treatment
  • Surgery if bowel necrosis, perforation or peritonitis
Gross description
  • Polypoid grape-like masses protruding through mucosa
Gross images

Images hosted on other servers:

Multiple subserosal bubbles

Diffuse hemorrhage

Pneumatosis intestinalis

Pneumatosis intestinalis and focal mucosal necrosis

Microscopic (histologic) description
  • Empty submucosal cysts lined by multinucleated giant cells
  • Mucosa may demonstrate cryptitis, crypt abscesses, granulomas
  • Can also resemble lipomatosis
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Mural pneumatosis

Mucosal pneumatosis

Videos

Pneumatosis intestinalis

Sample pathology report
  • Descending colon, resection:
    • Segment of colon with numerous mural cystic spaces lined by foreign body giant cells, consistent with pneumatosis cystoides intestinalis
    • Margins of resection unremarkable.
    • Four benign lymph nodes.
Differential diagnosis
  • Crohn's disease:
    • Granulomas and inflammation may somewhat mimic Crohn’s but there would not be air filled spaces
  • Other granuloma forming diseases:
    • Limited biopsy material may raise this differential diagnosis but most other granuloma forming diseases would show true granulomas rather than numerous multinucleated giant cells (Am J Surg Pathol 2007;31:1469)
Board review style question #1

Which of the following is true about pneumatosis cystoides intestinalis?

  1. It can occur in children
  2. It can progress to malignancy
  3. The cystic spaces contain both air and fat
  4. Treatment is supportive rather than surgical
Board review style answer #1
A. It can occur in children

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