LONG-TERM OUTCOME AFTER NEONATAL MECONIUM OBSTRUCTION

Objective. It is unclear whether children with cystic fibrosis (CF) wh o present with neonatal meconium ileus have a different long-term outc ome from those presenting later in childhood with pulmonary complicati ons or failure to thrive. We examined a cohort of patients with meconi um ileus, and compared their longterm outcome with children who had CF without meconium ileus and neonates who had meconium obstruction with out CF (meconium plug syndrome). Study Design. Comparative study using retrospective and follow-up interview data. Patients. Group 1 consist ed of 35 surviving CF patients who presented with meconium ileus betwe en 1966 and 1992. Two control groups were also studied: 35 age- and se x-matched CF patients without meconium ileus (group 2), and 12 infants presenting with meconium plug syndrome during the same time period (g roup 3). Outcome Measures. Pulmonary, gastrointestinal, nutritional, a nd functional status were reviewed, and surgical complications were re corded. Results. Mean follow-up was 12.6 +/- 6, 12.6 +/- 6, and 9.3 +/ - years in groups 1, 2, and 3,respectively. Patients without CF (group 3) demonstrated better growth and functional status, and had a lower incidence of pulmonary and gastrointestinal problems. Although the pre sence of meconium ileus among CF patients was associated with an earli er diagnosis, there were no significant differences between groups 1 a nd 2 with respect to hepatobiliary, nutritional, functional, or respir atory status. Meconium ileus was associated with a higher risk of meco nium ileus equivalent (20% vs 6%), although this difference was not st atistically significant. Long-term surgical complications (adhesive sm all bowel obstruction and blind loop syndrome) were seen in 27% of chi ldren with meconium ileus; there were no long-term surgical complicati ons in groups 2 or 3, because these infants did not have any neonatal surgical procedures. Children presenting with complicated meconium ile us had a higher rate of long-term surgical complications than those wi th uncomplicated meconium ileus (36% vs 17%), and those managed with r esection or enterostomy had more complications than those treated by e nterotomy and lavage (33% vs 0%). Conclusions. Long-term outcome is si milar in CF patients who present with meconium ileus and those who do not, except for a slightly higher incidence of meconium ileus equivale nt, and a significantly higher rate of surgical complications. The ris k of surgical complications is highest in those presenting with compli cated meconium ileus and those undergoing resection or enterostomy. Pa tients with meconium obstruction who do not have CF have an excellent long-term prognosis. This information will be useful in counseling the families of infants presenting with neonatal meconium obstruction.