Exomphalos major: the Northern Ireland experience

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.