Purpose or Case Report: Giant omphaloceles are large in size and contain a significant portion of liver. Giant omphaloceles are often associated with other co-morbidities, such as pulmonary hypoplasia, which can lead to respiratory insufficiency, prolonged intensive care support, assisted ventilation, and death. The purpose of this study is to develop a prognostic model for prediction of post-natal outcomes in patients with giant omphaloceles using fetal MRI calculated observed to expected total lung volumes (O/E TLV).
Methods & Materials: After IRB approval, a retrospective search was performed to identify patients with giant omphalocele who underwent fetal MRI and received pre- and post-natal care at our institution from 2007 to 2017. Patients with other anterior abdominal wall defects, including OEIS complex, were excluded. After review of the fetal MR images, 3D lung volumes and O/E TLV were calculated. Statistical analysis was performed using Chi-square and Student’s t-test.
Results: 14 patients met our inclusion criteria. Two of these patients died shortly after birth and two died in utero (one due to termination of pregnancy). 4 were female and 9 were male (1 was non-identified). Mean gestational age at fetal MRI was 26.5 ± 5.49 weeks with a mean O/E TLV of 0.56 ± 0.37. Mean gestational age at birth was 37.2 ± 1.89 weeks. O/E TLV did not significantly correlate with sex (p=0.53), pulmonary hypertension (p=0.69), need for tracheostomy (p=0.92), need for supplemental oxygen at discharge (p=0.16), or incidence of chronic lung disease (p=0.13). O/E TLV approached significance with regard to fetal distress at birth (p=0.06) and number of days intubated (p=0.057). O/E TLV did significantly correlate with the incidence of hypoxia at birth (p=0.005) and in-utero or neonatal death (p=0.004). Infants with hypoxia at birth had a mean O/E TLV of 0.53 ± 0.25. All infants with O/E TLV of less than 0.23 died in utero or around time of birth.
Conclusions: Fetal MRI calculated O/E TLV ratio may be helpful in predicting respiratory prognosis and survival in patients with giant omphalocele.