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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: | ||
| 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: | ||
| 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 This link will directly take you to the relevant new literature Pneumatosis Cystoides Intestinalis
| Synopsis by: Martin Nadel M.D. (T67000M34000)[336]
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