Nutritional Management for Infants with Cystic Fibrosis Born with Meconium Ileus: A 15-Year Review

Background/Objectives: Meconium ileus (MI) related to cystic fibrosis (CF) presents unique nutrition challenges for newborns. The aim of this study was to present a descriptive case series reviewing the management practice and clinical outcomes of infants with CF and MI at a large, quaternary care center. Methods: A retrospective analysis examined patients born with CF and MI over fifteen years at a large pediatric hospital in the Midwest. Patient demographics, prenatal imaging data, newborn screening results, stool studies, sweat chloride and details of the clinical course were obtained through the electronic medical record (EMR). Outcomes including length of stay (LOS), timing of surgery, and duration of total parenteral nutrition (TPN) needs were compared between infants with and without a surgical ostomy. Associations of MI severity with CF genotype, stool elastase, and prenatal imaging findings were evaluated. Results: A total of 30 neonates (21 female, 9 male) were included. Surgery was required in 26 infants, with 18 requiring ostomy placement. There were no significant correlations between CF genotype, stool elastase, or prenatal imaging findings with need for ostomy placement. Compared to those without ostomy placed, infants with ostomies had significantly longer median [IQR] LOS (71 [61, 105] vs. 22 [16, 32] days; difference 49, 95% CI 34-77) and duration of TPN (52 [41, 74] vs. 8 [6, 12] days; difference 43, 95% CI 25-62) (p < 0.001 for both). Conclusions: This work highlights the complex medical needs of these infants, requiring prolonged hospitalization and TPN for adequate nutrition. We share our institution's approach to the nutritional management infants with CF and MI, with a focus on collaboration needed with the multidisciplinary team.