Eosinophilic infiltration of the enteric neural plexuses in Hirschsprung's disease

Inflammatory infiltrations of the enteric plexuses are uncommon and are usually lymphoplasmacytic. Within the past 15 years, nine pediatric cases in which a predominantly eosinophilic infiltrate of the gastrointestinal wall with a predilection for the myenteric (Auerbach's) and deep submucosal (Henle's) plexuses were seen at our institution. Two were neonates without gastrointestinal abnormalities who expired shortly after birth. Seven were patients with short-segment Hirsch-sprung's disease. There was a mild increase in mucosal eosinophils in the overlying mucosa and only one patient had peripheral eosinophilia. Follow-up data obtained 1 month to 7 1/2 years after biopsy revealed no development of inflammatory bowel disease, connective tissue disease, malignancy, allergic disorder, or intestinal dysmotility. The proximal location of the infiltrate suggests that it may represent a secondary finding rather than a primary cause of aganglionosis.