Patients with congenital cardiac disease and left-ventricular outflow tract obstruction are at high risk of necrotizing enterocolitis. However, non–IgE-mediated gastrointestinal food-induced allergic disorders might present themselves with a similar clinical picture and we believe that these clinical entities have been underestimated and could be misinterpreted as necrotizing enterocolitis. In our analysis, we highlight the increased incidence of food protein–induced enterocolitis syndrome and allergic proctocolitis, discuss their possible pathophysiological pathways and their impact on morbidity and clinical care in this patient group.
Methods and results
Twenty-four patients with left-ventricular outflow tract obstruction were included in the analysis. Six patients (25%, 3 female) showed symptoms (onset at 33 ± 21 days of age) compatible with food protein-induced allergic proctocolitis or enterocolitis syndrome. Clinical picture was inconsistent with classical necrotizing enterocolitis, nevertheless three patients received conservative treatment for a necrotizing enterocolitis. Blood cultures, C-reactive protein (<4 mg/L ± 0) and leucocytes (9.1 ± 1G/L) were negative but relevant eosinophilia (16.6 ± 6.9%) was present in five patients. After elimination of cow milk all patients experienced symptoms resolution in 5 ± 2 days.
Non-IgE-mediated gastrointestinal food-induced allergic disorders can mimic necrotizing enterocolitis and need to be taken in account in well-appearing neonates with congenital heart disease and left-ventricular outflow tract obstructions presenting atypical necrotizing enterocolitis symptoms. The partially insufficient perfusion of the bowel wall due to left-ventricular outflow tract obstruction can enhance bacterial translocation and seems to be responsible for an aberrant immune response to ingested milk protein. Improving the systemic perfusion with an earlier relief of the obstruction of the left-ventricular outflow tract should be aimed for. To protect the homeostasis of the intestinal flora hydrolyzed or amino acid-based formula milk together with complementary or prophylactic strategies (such as stimulating intestinal motility or treating intestinal bacterial overgrowth) should be used.