{
  "abstract": "Exomphalos occurs in 1/13000 live births, and is a central abdominal wall defect, allowing herniation of abdominal viscera, covered by a membrane, into the umbilical cord. Exomphalos can be defined as major or minor dependent on defect size and liver herniation, 1 and is associated with comorbidities in up to 72% of cases.2 Literature on long term respiratory and nutritional outcomes associated with exomphalos is limited, compared to gastroschisis, another abdominal wall defect.3–5 This study aimed to examine the longterm nutritional and respiratory outcomes in a single centre cohort of infants with exomphalos.A retrospective cohort study of respiratory and nutritional outcomes of babies born with exomphalos in a single tertiary neonatal unit between 2000 – 2020 was carried out. Babies were identified from the clinical coding database and all infants with an abdominal wall defect were identified. Babies with gastroschisis were excluded. Case notes were reviewed and data collected on demographic details, other comorbidities, surgical management, nutritional and respiratory outcomes and mortality. For comparative purposes cases were divided into exomphalos major and minor. Relative risk (RR) with 95% confidence intervals and p values were calculated with an alpha of <0.05 considered significantly different.Gender of infants and median gestational age at delivery was similar between groups. Preterm births are similar in both groups with a higher incidence (40% vs 3%) of postnatal diagnosis in infants with examphalos minor. 48% of infants with exomphalos minor and 75% with major had other comorbidities. Median length of stay was 6 days in minor versus 43 days in major. Mean length of invasive ventilation was 4 days in minor compared to 13 days in major. 1(3%) infant with major required home oxygen, and 5 (14%) infants with major required tracheostomies. 1(4%) infant with minor required parenteral nutrition (PN) compared to 10 (28%) with major. Median days to reach full feeds was 4 days for infants with minor and 8 days for infants with major. All surviving infants with minor were orally fed at age 3 compared to 67% of children with major. Duration of parenteral nutrition (PN) was longer in major compared to minor. No children required home PN.Infants with exomphalos major had longer hospital stays and higher rates of comorbidities and complications. Exomphalos minor was not associated with long term respiratory or nutritional consequences. 5 (14%) children with exomphalos major required tracheostomy, and 8 (45%) required long term enteral feeding support – all had significant comorbidities. No children required home PN.This data offers insight into likely long term outcomes for infants with exomphalos. This is useful for developing departmental practices and for parents as part of the antenatal or postnatal counselling process, but further research as part of multi-centre networks is needed.References Poddar R, Hartley L. Exomphalos and gastroschisis. Continuing Education in Anaesthesia Critical Care & Pain 2009;9(2):48–51.Carachi R, Doss S. Clinical Embryology: An Atlas of Congenital Malformations. Springer; 2019. 304–305 p.Vachharajani AJ, Rao R, Keswani S. Outcomes of exomphalos: an institutional experience. Pediatric Surgery International 2009;25:139–44.Raymond SL, Hawkins RB, Peter SDS, Downard CD, Qureshi FG, Renaud E, et al. Predicting morbidity and mortality in neonates born with gastroschisis. Journal of Surgical Research 2020;245:217–24.Vinit N, Talbotec C, de Tristan MA, Salomon LJ, Giuseppi A, Rousseau V, et al. Predicting factors of protracted intestinal failure in children with gastroschisis. The Journal of Pediatrics 2021;243:122–9.",
  "authors": [
    {
      "affiliations": [
        "Department of Paediatric Gastroenterology, Royal Manchester Children’s Hospital"
      ],
      "name": "Sian Copley"
    },
    {
      "affiliations": [
        "Department of Paediatric Surgery, Royal Victoria Infirmary, Newcastle upon Tyne. NE1 4LP"
      ],
      "name": "Alok Godse"
    },
    {
      "affiliations": [
        "Department of Obstetrics, Royal Victoria Infirmary, Newcastle upon Tyne. NE1 4LP"
      ],
      "name": "Gareth Waring"
    },
    {
      "affiliations": [
        "Department of Paediatric Surgery, Royal Victoria Infirmary, Newcastle upon Tyne. NE1 4LP"
      ],
      "name": "Gareth Hosie"
    },
    {
      "affiliations": [
        "Department of Neonatology, Royal Victoria Infirmary, Newcastle upon Tyne. NE1 4LP"
      ],
      "name": "Elda Dermyshi"
    },
    {
      "affiliations": [
        "Department of Neonatology, Royal Victoria Infirmary, Newcastle upon Tyne. NE1 4LP"
      ],
      "name": "Naveen Kumar Athiraman"
    }
  ],
  "title": "OC102 Clinical outcomes of infants born with exomphalos: 20-year experience from a UK regional institution",
  "uid": "3a43f935-e187-59ea-acda-2e83cdab1823"
}