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A routine antenatal ultrasound scan in the first trimester revealed a large omphalocele mainly occupied by liver in a female infant. Owing to preterm labour she was delivered by caesarean section at 29+4 weeks of gestation with a birth weight of 1450 g. No other malformations were present and her karyotype was normal.
The neonatal period was complicated by severe respiratory distress with pulmonary hypertension requiring prolonged high-frequency ventilation and inhaled nitric oxide. The infant developed bilateral grade 2 intraventricular haemorrhages. A haemodynamically significant ductus arteriosus was closed surgically. Owing to these complications, primary closure of the 4×4 cm large omphalocele (figure 1) was initially not feasible. Palliative care was considered, but was not …
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