Long-term respiratory support in children with giant omphalocele

Omphalocele is one of the most common fetal abdominal wall defects. When this defect is of giant size, significant respiratory compromise may occur and impact on prognosis. We present three infants with giant omphalocele, highlighting the potential need for ongoing ventilatory support after the neonatal period in children born with this condition. The three cases had very different outcomes but all had significant ventilatory insufficiency and required substantial respiratory support at least into the second year of life. The possibility of a requirement for long-term ventilatory support should be discussed with families at antenatal diagnosis. A conservative surgical approach, together with early monitoring for hypoventilation and screening for the development of pulmonary hypertension is indicated for these children to limit morbidity. We suggest early tertiary respiratory input and advocate for a specific case manager to oversee the regional care of these children.