Pigbel of the Stomach – A Rare Cause of Fulminant Gastric Necrosis

Abstract

Enteritis necroticans (pigbel) is a segmental necrotizing infection of the jejunum and ileum caused by Clostridium perfringens (C.p.) type C. The disease occurs sporadically in developing countries and is extremely rare in the U.S. We present what we believe is the first report in the U.S. of pigbel involving the stomach in a diabetic adult. An 80 year old diabetic man presented with a several hour history of lower chest pain and indigestion. His initial exam was entirely benign without rebound tenderness or guarding. Laboratory values included: WBC 9.8, Hct 31, platelets 257. Cardiac enzymes were normal. A non-contrasted CT of the abdomen revealed gas in the wall of the stomach, the gastric veins and the portal vein. A pre-operative endoscopic evaluation showed an impressive black discoloration of the mucosa in the proximal half of the stomach. Antrum and duodenum appeared normal. The patient underwent a subtotal gastrectomy with Roux-en-Y gastrojejunostomy. No obvious vascular occlusions were detected at the time of surgery. The histologic evaluation confirmed complete necrosis of the proximal gastric wall and a large number of gram-positive rods with the characteristic appearance of C.p. The patient developed persistent fevers and multiorgan failure. He expired 4 days postoperatively. Enteritis necroticans was first described in Germany in the 1940s as “Darmbrand” (literally “bowel fire”) in malnourished people who became ill after consuming large quantities of rancid meat. The term “pigbel” was used when the same codition re-emerged in the 1960s in Papua New Guinea where it was often associated with consumption of poorly cooked pork by protein-deficient persons in ceremonial feasts. C.p. was cultured from the intestinal lumen, and the beta-toxin produced by the organism during the phase of rapid growth was shown to be the mediator of intestinal necrosis. While C. p. may be a constituent of normal bowel flora, the beta-toxin is extremely sensitive and is usually destroyed by gastric acid or proteolysis. In the U.S., few cases of pigbel have been described, principally in diabetics and the small bowel or colon is typically affected. To our knowledge, our case represents the first example of pigbel involving the stomach. Despite a very high mortality, survival has been described with antibiotic tx or surgery. Although extremely rare, enteritis necroticans should be considered in the Ddx of diabetic patients presenting with an acute abdomen or bloody diarrhea.