Mortality and short-term morbidity in infants with exomphalos

TY - JOUR

T1 - Mortality and short-term morbidity in infants with exomphalos

AU - Sakonidou, Susanna

AU - Ali, Kamal

AU - Farmer, Isabel

AU - Hickey, Ann

AU - Greenough, Anne

PY - 2018/5

Y1 - 2018/5

N2 - Background Infants with exomphalos major have a high mortality and morbidity. The aims of this study were to identify predictors of survival regardless of the size of the exomphalos, and to analyze morbidity in infants with exomphalos minor.Methods Patients were classified as having exomphalos major or minor based on whether the liver was in the exomphalos sac, and the size of the abdominal wall defect. The respiratory, gastrointestinal and surgical outcomes of 50 infants with exomphalos (including 27 with exomphalos major) were assessed. Receiver operating characteristic (ROC) curves were constructed to identify factors predictive of survival. Results No infant with exomphalos minor died; there were seven deaths in the exomphalos major group (P < 0.001). Infants with exomphalos minor who had chromosomal abnormalities (six had a genetic diagnosis of Beckwith–Wiedemann syndrome) developed severe respiratory distress or chronic respiratory morbidity. Nasogastric feeding at discharge was required in 37% of infants with exomphalos major and in 17% with exomphalos minor. Lower gestational age (area under the ROC curve [AUROC], 0.814) and birthweight (AUROC, 0.797), and longer duration of ventilation (AUROC, 0.853) and of supplementary oxygen (AUROC, 0.810) were predictive of mortality. Conclusions Infants with exomphalos regardless of size can have chronic morbidity. Mortality is commonest in those with exomphalos major born at lower gestational age and birthweight.

AB - Background Infants with exomphalos major have a high mortality and morbidity. The aims of this study were to identify predictors of survival regardless of the size of the exomphalos, and to analyze morbidity in infants with exomphalos minor.Methods Patients were classified as having exomphalos major or minor based on whether the liver was in the exomphalos sac, and the size of the abdominal wall defect. The respiratory, gastrointestinal and surgical outcomes of 50 infants with exomphalos (including 27 with exomphalos major) were assessed. Receiver operating characteristic (ROC) curves were constructed to identify factors predictive of survival. Results No infant with exomphalos minor died; there were seven deaths in the exomphalos major group (P < 0.001). Infants with exomphalos minor who had chromosomal abnormalities (six had a genetic diagnosis of Beckwith–Wiedemann syndrome) developed severe respiratory distress or chronic respiratory morbidity. Nasogastric feeding at discharge was required in 37% of infants with exomphalos major and in 17% with exomphalos minor. Lower gestational age (area under the ROC curve [AUROC], 0.814) and birthweight (AUROC, 0.797), and longer duration of ventilation (AUROC, 0.853) and of supplementary oxygen (AUROC, 0.810) were predictive of mortality. Conclusions Infants with exomphalos regardless of size can have chronic morbidity. Mortality is commonest in those with exomphalos major born at lower gestational age and birthweight.

U2 - 10.1111/ped.13537

DO - 10.1111/ped.13537

M3 - Article

SN - 1328-8067

JO - PEDIATRICS INTERNATIONAL

JF - PEDIATRICS INTERNATIONAL

ER -