FIBROSING COLONOPATHY IN CHILDREN WITH CYSTIC-FIBROSIS

Purpose: Fibrosing colonopathy is a newly described entity seen in chi ldren with cystic fibrosis. The radiological hallmarks are foreshorten ing of the right colon with varying degrees of structure formation. Hi gh-dose enzyme therapy has been implicated as the cause of this proces s. The purpose of this study is to review the author's experience with evaluation and treatment of these patients. Methods: There are curren tly 380 patients being treated at our CF center. Fifty-five of these p atients have been treated with high-dose enzyme therapy (> 5,000 units of lipase/kg). The medical records of these patients, who are at risk for developing fibrosing colonopathy, were reviewed for the presence of recurrent abdominal complaints, and the work-up and treatment of th ese symptoms. Results: Chronic complaints of abdominal pain, distensio n, change in bowel habits, or failure to thrive were present in 24 of the 55 patients treated with high-dose enzymes. So far, 18 of these 24 patients have been evaluated by contrast enema. Thirteen of eighteen have been found to have fibrosing colonopathy characterized by foresho rtening and structures of the colon. Additional findings included foca l strictures of the right colon (7 of 13), long segment strictures (5 of 13), and total colonic involvement (1 of 13). Nine patients with th e most severe symptoms have undergone colon resection, including five segmental right colectomies, three extended colectomies (ileo-sigmoid anastomosis), and one subtotal colectomy with end-ileostomy. pathologi cal evaluation has shown submucosal fibrosis, destruction of the muscu laris mucosa, and eosinophilia. No postoperative complications or deat hs occurred. All nine postoperative patients have noted marked symptom atic improvement. Contrast enema follow-up results are available for s ix patients, and have documented no recurrent strictures to date. Thre e of four nonoperative patients have less severe symptoms and are curr ently being treated conservatively. The other family has refused surge ry and the patient is being treated symptomatically. Conclusion: High- dose lipase replacement has been implicated as the etiology for FC and was present in all of our patients. Our cystic fibrosis center now ro utinely limits lipase to 2,500 U/kg per dose. We recommend the use of contrast enemas to evaluate at-risk patients who have chronic abdomina l complaints or who present with recurrent bowel obstruction. Colon re section should be performed in those with clinically and radiographica lly significant strictures with the expectation of a good outcome. Cop yright (C) 1997 by W.B. Saunders Company.