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Pneumatosis Cystoides Intestinalis
Etiology

Two forms of pneumatosis exist. Primary pneumatosis (15% of cases) is of unknown etiolgy. Secondary causes (85%) fall into two broad categories, those associated with obstructive pulmonary disease and those with necrotizing intestinal disease.
The primary form affects primarily the colon, while the secondary form affects the small intestine.
The scondary form has been associated with obstructive pulmonary and obstructive pyloric-duodenal disease, and with necrotizing intestinal diseases, the two most common being necrotizing enterocolitis and Idiopathic Inflammatory Bowel Disease.
Pathogenesis

Three mechanisms of formation have been postulated.
The first is mechanical trapping of air along tissue planes due to rupture of blebs in obstructive lung disease. The second is invasion of devitalized bowel wall by gas forming bacteria, and the third is excess production of luminal gas by fermentation of carbohydrates with absorption and trapping of the air in the bowel wall.,
Epidemiology

There is a slightly higher incidence in children due to the occurrence of pneumatosis associated with necrotizing enterocolitis.
Other than young children no definite age, sex or geographic predominance is known.
General Gross Description

The appearance is the same in the large and small intestine.
The mucosa is raised by smooth surfaced soft blebs or flatter plaques.
Cysts are diffusely distributed and vary from several miilimeters to several centimeters.
Cysts may mimic the appearance of lymphangiomas and can have a polypoid appearance.
•Examples:
Pneumatosis Intestinalis
General Microscopic Description

The cysts are air filled and found primarily in the submucosa.
A multinucleated foreign body giant cell reaction may be present and inflammatory changes including fibrosis may occur.
•Examples:
Clinical Correlation

Pneumatosis is usually discovered as an incidental finding or may present a vague low grade abdominal discomfort.
The condition may resolve spontaneously within several weeks with resorption of the gas but may on occasion recur.
Attempts to increase the rate of absorption have included oxygen therapy and hyperbaric oxygen, but the primary therapy is directed towards any underlying disease.
References

Sleisenger MH, Fordtran JS. Gastrointestinal disease. 5th ed. Philadelphia: Saunders, 1993, pp. 1542-1545
Gastrointestinal Pathology, Fenoglio-Preiser C.M. et al, New York: Raven Press, 1989, pp. 687-690

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Pneumatosis Cystoides Intestinalis
Synopsis by: Martin Nadel M.D. (T67000M34000)[336]
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