The meconium ileus equivalent in mucoviscidosis

Abstract

Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of 120 CF patients, is analyzed. 9 were successfully managed by medical treatment alone, 1 three months old infant was treated surgically with no later relapse and a 26 years old patient with a chronic deleterious course leading to opiate dependency could be relieved only by ileostomy. 5 patients experienced only 1 episode of MIE, 2- two to three and in 4 a chronic refractory course with multiple episodes was probably due to an inadequate medical management. The evaluation of the events in our patients and of the available data from the literature allows the following conclusions: MIE is a preventable condition in CF patients; a rigorous medical treatment of exocrine pancreatic insufficiency supplemented with mucolytic agents orally can usually prevent MIE and relieve an established MIE; surgical treatments is indicated only in desperate situations.