COLONIC STRICTURES IN CHILDREN WITH CYSTIC-FIBROSIS

PURPOSE: To determine the radiographic, clinical, surgical, and histol ogic findings in children with cystic fibrosis who develop strictures of the colon. MATERIALS AND METHODS: Ten children (five boys, five gir ls; age range, 2.5-9.0 years; mean age, 5.5 years), who were treated a t the practices of the authors, were retrospectively identified and th eir medical records reviewed. RESULTS: Radiographic manifestations of the colonic disease included mucosal irregularity and spiculation with nodular thickening of the colonic wall and loss of normal colonic hau stration. Luminal narrowing involved long segments of the colon. Longi tudinal shortening of the colon was also a prominent feature. The decr ease in caliber of the bowel ranged from mild narrowing to complete oc clusion of the lumen. Histologic examination revealed severe submucosa l fibrosis and fatty infiltration with transmural extension of the fib rosis to involve the serosa in some cases. Unlike in Crohn disease, ho wever, acute inflammatory changes were minimal or absent. CONCLUSION: Colonic stricture in children with cystic fibrosis is due to irreversi ble and frequently progressive narrowing of the colonic lumen.