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Exomphalos major: the Northern Ireland experience
P Charlesworth1, E Ervine, M McCullagh
1The Royal Alexandra Hospital for Sick Children, Brighton, UK. paul.charlesworth@bsuh.nhs.uk
Insights
Exomphalos major (EM) can be successfully managed non-operatively, enabling early feeding and discharge. This approach allows granulation before ventral hernia repair, with a 46% mortality rate in this study.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Anomalies
Background:
- Exomphalos major (EM) presents significant challenges in abdominal wall defect closure.
- Effective management strategies for EM are crucial for infant survival and outcomes.
Purpose of the Study:
- To evaluate the single-center experience and outcomes of treating exomphalos major.
- To assess the efficacy of non-operative management for EM.
Main Methods:
- A 15-year retrospective review of case notes for infants with exomphalos major.
- Data analysis focusing on treatment approaches, complications, and outcomes.
Main Results:
- Fourteen infants with EM were identified; 46% mortality rate among live births.
- Severe pulmonary hypoplasia occurred in 54% of infants.
- Non-operative management was primary, with silver sulphadiazine application and early enteral feeding.
Conclusions:
- Non-operative management of exomphalos major is a viable approach, facilitating early enteral feeding and discharge.
- This strategy allows for granulation and subsequent ventral hernia repair.
- High rates of mortality and pulmonary hypoplasia underscore the severity of EM.
Purpose:
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.
Materials:
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.
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