Nutritional Intake, Energy Expenditure, and Growth of Infants Following Congenital Diaphragmatic Hernia Repair :

To examine factors influencing the adequacy of energy and protein intake in the pediatric intensive care unit and to describe their relationship to clinical outcomes in mechanically ventilated children. We conducted an international prospective cohort study of consecutive children (ages 1 month to 18 yrs) requiring mechanical ventilation longer than 48 hrs in the pediatric intensive care unit. Nutritional practices were recorded during the pediatric intensive care unit stay for a maximum of 10 days, and patients were followed up for 60 days or until hospital discharge. Multivariate analysis, accounting for pediatric intensive care unit clustering and important confounding variables, was used to examine the impact of nutritional variables and pediatric intensive care unit characteristics on 60-day mortality and the prevalence of acquired infections. 31 pediatric intensive care units in academic hospitals in eight countries participated in this study. Five hundred patients with mean (SD) age 4.5 (5.1) yrs were enrolled and included in the analysis. Mortality at 60 days was 8.4%, and 107 of 500 (22%) patients acquired at least one infection during their pediatric intensive care unit stay. Over 30% of patients had severe malnutrition on admission, with body mass index z-score >2 (13.2%) or <-2 (17.1%) on admission. Mean prescribed goals for daily energy and protein intake were 64 kcals/kg and 1.7 g/kg respectively. Enteral nutrition was used in 67% of the patients and was initiated within 48 hrs of admission in the majority of patients. Enteral nutrition was subsequently interrupted on average for at least 2 days in 357 of 500 (71%) patients. Mean (SD) percentage daily nutritional intake (enteral nutrition) compared to prescribed goals was 38% for energy and 43% (44) for protein. A higher percentage of goal energy intake via enteral nutrition route was significantly associated with lower 60-day mortality (Odds ratio for increasing energy intake from 33.3% to 66.6% is 0.27 [0.11, 0.67], p = .002). Mortality was higher in patients who received parenteral nutrition (odds ratio 2.61 [1.3, 5.3], p = .008). Patients admitted to units that utilized a feeding protocol had a lower prevalence of acquired infections (odds ratio 0.18 [0.05, 0.64], p = .008), and this association was independent of the amount of energy or protein intake. Nutrition delivery is generally inadequate in mechanically ventilated children across the world. Intake of a higher percentage of prescribed dietary energy goal via enteral route was associated with improved 60-day survival; conversely, parenteral nutrition use was associated with higher mortality. Pediatric intensive care units that utilized protocols for the initiation and advancement of enteral nutrient intake had a lower prevalence of acquired infections. Optimizing nutrition therapy is a potential avenue for improving clinical outcomes in critically ill children.

Infants with congenital diaphragmatic hernia often require intensive treatment after birth, have prolonged hospitalizations, and have other congenital anomalies. After discharge from the hospital, they may have long-term sequelae such as respiratory insufficiency, gastroesophageal reflux, poor growth, neurodevelopmental delay, behavior problems, hearing loss, hernia recurrence, and orthopedic deformities. Structured follow-up for these patients facilitates early recognition and treatment of these complications. In this report, follow-up of infants with congenital diaphragmatic hernia is outlined.

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity, although the underlying mechanism has not been fully understood yet. In adult CDH survivors incidence of esophagitis is high and even Barrett's esophagus may ensue. Yet, in many CDH patients a clinical history compatible with GERD seems to be lacking, which may result in missing patients with pathologic reflux disease. Prolonged unrecognized GERD may eventually result in failure to thrive. This has been found in many young CDH patients, which may also be caused by insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. In patients treated with ECMO, the incidence of neurological deficits is even higher, probably reflecting more severe hypoxemia and the risk of ECMO associated complications. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but most of them concentrate on the first years after repair and only a few studies focus on the health-related quality of life in CDH patients. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are mandatory. Based on such studies a more focused approach for routine follow-up programs may be established.