title: Exomphalos major: the Northern Ireland experience.
abstract: OBJECTIVE
  In exomphalos major (EM), closure of the defect in the abdominal wall presents a challenge. The aim of this study is to evaluate a single centre experience of EM.
  
  
  METHODS
  A 15-year retrospective case-note review; data presented as median (range).
  
  
  RESULTS
  Fourteen infants (7 female) were born with EM: birth weight 2.9 (1.2-3.8) kg, gestational age 38 (31-39) weeks. One infant died in utero and one within the first hour of life. Severe pulmonary hypoplasia was present in 7/13 (54%), and there was a mortality of 6/13 (46%) live births. Infants were treated non-operatively primarily. Two infants underwent early surgery: one infant, born with a ruptured sac, had a surgical silo constructed on day 1 and closure on day 8, while a second infant had partial closure (skin only) on day 11. Ten infants had application of silver sulphadiazine to the sac 2-3 times per week. Enteral feeds were established soon after birth. They were discharged from hospital to allow granulation. Ventral hernia closure was performed on a subsequent admission.
  
  
  CONCLUSIONS
  Exomphalos major can be successfully treated non-operatively, allowing immediate enteral feeding and early discharge while granulation takes place. In this series, exomphalos major has an incidence of 1 in 26,000, mortality is 46% and severe pulmonary hypoplasia is present in 54% of infants.
authors.affiliation: The Royal Alexandra Hospital for Sick Children, Brighton, UK. paul.charlesworth@bsuh.nhs.uk
authors.forename: P
authors.initials: P
authors.lastname: Charlesworth
authors.forename: E
authors.initials: E
authors.lastname: Ervine
authors.forename: M
authors.initials: M
authors.lastname: McCullagh
mesh_terms: D000891:Anti-Infective Agents, Local / Q000627:therapeutic use; D001458:Bandages; D004750:Enteral Nutrition; D005260:Female; D005313:Fetal Death; D006554:Hernia, Umbilical / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D007494:Ireland / Q000453:epidemiology; D007902:Length of Stay / Q000706:statistics & numerical data; D008168:Lung / Q000002:abnormalities; D008297:Male; D011247:Pregnancy; D012189:Retrospective Studies; D012837:Silver Sulfadiazine / Q000627:therapeutic use
chemical_list: D000891:Anti-Infective Agents, Local; D012837:Silver Sulfadiazine