Susanna Sakonidou1, Kamal Ali1, Isabel Farmer1, Ann Hickey1, Anne Greenough1,2,3,4. 1. Neonatal Intensive Care Centre, King's College Hospital NHS Foundation Trust, London, UK. 2. MRC and Asthma UK Centre in Allergic Mechanisms of Asthma, King's College London, London, UK. 3. Women and Children's Health, School of Life Sciences, Faculty of Life Sciences and Medicine, King's College London, London, UK. 4. NIHR Biomedical Research Centre at Guy's and St Thomas', NHS Foundation Trust, King's College London, London, UK.
Abstract
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.
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.
Authors: Wendy N Nembhard; Jorieke E H Bergman; Maria D Politis; Jazmín Arteaga-Vázquez; Eva Bermejo-Sánchez; Mark A Canfield; Janet D Cragan; Saeed Dastgiri; Hermien E K de Walle; Marcia L Feldkamp; Amy Nance; Miriam Gatt; Boris Groisman; Paula Hurtado-Villa; Kärin Kallén; Danielle Landau; Nathalie Lelong; Jorge Lopez-Camelo; Laura Martinez; Margery Morgan; Anna Pierini; Anke Rissmann; Antonin Šípek; Elena Szabova; Giovanna Tagliabue; Wladimir Wertelecki; Ignacio Zarante; Marian K Bakker; Vijaya Kancherla; Pierpaolo Mastroiacovo Journal: Birth Defects Res Date: 2020-10-17 Impact factor: 2.661