Background: Long-term negative sequelae of esophageal atresia (EA) may induce poor growth and impaired nutritional status in childhood. We describe the nutritional profile and energy metabolism of children with repaired EA to identify malnutrition risk factors and optimize growth management. Methods: Twenty-one children (>4 years) were included, and anthropometric measurements, nutritional assessment, and energy metabolism were considered. The subjects were defined as undernourished if they met BMI < −2 standard deviation (SD). To grade undernutrition, we defined the prevalence of underweight, stunting, and wasting (cut-off level of <−2 SD). Medical records were reviewed for the type of EA and surgery and perinatal data. Results: Malnutrition was detected in 28.6% of children. Underweight was detected in 23.8% of patients (all with undernutrition
p < 0.01). Wasting was noted in 28.6% of patients, of these 5 children were undernourished (
p < 0.001) and stunting was noticed in only one patient with malnutrition (
p = 0.5). Resting expenditure energy (REE) was lower in undernourished subjects compared to subjects with adequate nutritional status (
p < 0.001). Malnutrition was associated to: type of EA (
p = 0.003, particularly type A and C); intervention including deferred anastomosis due to long-gap repair (
p = 0.04) with/or without jejunostomy (
p = 0.02), gastric pull-up (
p = 0.04), primary anastomosis (
p = 0.04), pyloromyotomy in long-gap (
p < 0.01); small for gestational age condition (
p = 0.001). Conclusions: undernutrition risk factors, beyond the type of malformation, surgery, and perinatal factors, must be early considered to personalize nutritional programming. Energy metabolism is important to monitor the nutritional requirements. The management of nutritional issues is surely a contributory factor able to counteract the poor growth of children with EA.
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