A case series of successfully managing exomphalos major with awake graduated compression dressing and early enteral feeding.

Abstract

Exomphalos anomaly is defined as the herniation of abdominal viscera into the base of the umbilical cord, with only a membranous sac covering these contents. It has an incidence of approximately 1 in 4000-6000 births. Management of exomphalos major (EM) remains controversial and limited, with very few studies to guide decision-making. This is a case series of four neonates with EM treated at a tertiary paediatric referral centre between 2018 and 2021 with a gradual compression dressing technique. Four neonates were diagnosed with EM. The average gestational age was 38 + 5 (range 38 + 2 - 39 + 2), and the average birth weight was 3.1kg (range 2.56 - 3.49kg). The defect size ranged between 5 and 7cm. All patients were commenced on gradual compression dressing between days 1 and 3 of life. Dressings were applied at the bedside in the general neonatal ward. The average time taken to reach full feeds was 1week; only one patient required parenteral nutrition. Three underwent surgical repair at two and 16weeks of age; one had delayed repair at the age of 1year because of the COVID-19 pandemic. None required patch repair. None required prolonged ventilation after repair. This case series describes a successful compression dressing technique that reduces sac content without the need for general anaesthetic or respiratory compromise, whereby simultaneous enteral feeding is tolerated.