Colon
Other nonneoplastic
Pneumatosis cystoides intestinalis
Author: Raul S. Gonzalez, M.D.
Last author update: 4 February 2021
Last staff update: 6 April 2023
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: pneumatosis cystoides intestinalis colon [title]
Table of Contents
Definition / general | Essential features | Sites | Etiology | Clinical features | Radiology description | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Videos | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Gonzalez RS. Pneumatosis cystoides intestinalis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/colonpneumatosis.html. Accessed April 19th, 2024.
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
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
- 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)
Additional references
Board review style question #1
Which of the following is true about pneumatosis cystoides intestinalis?
- It can occur in children
- It can progress to malignancy
- The cystic spaces contain both air and fat
- Treatment is supportive rather than surgical
Board review style answer #1
