Surgical management of infantile ulcerative enteritis

Infantile transmural ulcerative enteritis is a disorder of early infancy characterized by feeding difficulties, intermittent and progressive diarrhea, cachexia, anemia, abdominal distention, and small-bowel dilation which may progress to intestinal obstruction. The pathologic process, of unknown etiology, involves a transmural enteritis with deep undermining mucosal ulceration, not unlike that seen in Crohn's disease, except that granulomas are usually not present. The early stages of the diseases may be reversible if the bowel is simply placed at rest by use of intravenous nutrition. In the later stages of the illness, there is progressive mechanical and functional intestinal obstruction due to inflammatory constriction of the distal small bowel and lack of effective peristalsis through the inflammed segments. The terminal stages are characterized by marked abdominal distention, complete obstruction, septicemia, and death. It is during the period of abdominal distention due to progressive intestinal obstruction that surgical intervention is of benefit. A cutaneous enterostomy proximal to the involved segments of small intestine serves to decompress the bowel, to minimize bacteremia, and to allow the distal inflamed intestine to heal. Total intravenous nutrition is mandatory for a period of several weeks until there is healing of the distal small bowel and closure of the enterostomy. In all surviving infants, bowel function has returned to normal and there have been no long-term sequelae or recurrences.