Giant omphalocele associated pulmonary hypertension: A retrospective study

BACKGROUND: Omphalocele is a common congenital defect of the abdominal wall, management of giant omphalocele (GO) is particularly for pediatric surgeons and neonatologists worldwide. The current study aimed to review and summarize the clinical features and prognosis in neonates with GO complicated with pulmonary hypertension (PH), which is associated with increased mortality, while in hospital. MATERIALS AND METHODS: Medical records of infants with GO between July 2015 and June 2020 were retrospectively analyzed. The patients enrolled were divided into PH and non-PH groups based on the presence or absence of PH, and patients with PH were divided into death and survival groups based on survival status. Clinical characteristics and outcomes were compared between groups, respectively. The risk factors for PH were analyzed by binary logistic regression. RESULTS: In total, 67 neonates were identified as having GO and 24 (35.8%) were complicated with PH. Infants with PH were associated with intubation within 24 h after birth (p = 0.038), pulmonary dysplasia (p = 0.020), presence of patent ductus arteriosus (PDA; p = 0.028), a staged operation (p = 0.002), longer mechanical ventilation days (p < 0.001), oxygen requirement days (p < 0.001), parenteral nutrition (PN) days (p < 0.001), length of neonatal intensive care unit (NICU) or hospital stay (p = 0.001 and 0.002, respectively), and mortality (p = 0.001). The results of multivariable logistic regression analysis revealed that a staged operation was independently associated with PH. In addition, PH patients with lower birth weight, higher peak of pulmonary arterial systolic pressure, and refractory to pulmonary vasodilators (PVD) had increased mortality. CONCLUSION: Pulmonary hypertension is a serious complication and significantly increases the mortality and morbidities in infants with a GO. In addition, early and serial assessment of PH by echocardiography should be a routine screening scheme, especially in the neonatal omphalocele population who required a staged surgical repair. Clinicians should be aware that infants with PH who had low weight, severe and refractory PH have a higher risk of death.