Case 1: Preterm Neonate with Persistent Respiratory Distress Despite Interventions

A preterm male neonate is born at 31 weeks of gestation via cesarean section for a category 2 fetal heart tracing. The prenatal history is significant for maternal autosomal dominant polycystic kidney disease (ADPKD). The pregnancy had been complicated by maternal chronic hypertension, preeclampsia with severe features, and significant enlargement with increased echogenicity of fetal kidneys on ultrasonography. Maternal laboratory findings are reassuring and antenatal steroids complete before delivery. At delivery, the neonate has poor respiratory effort, requiring continuous positive airway pressure and is transferred to the NICU. On arrival, he is noted to be in respiratory distress with coarse breath sounds bilaterally, grunting, and significant retractions. Evaluation for infection is initiated and antibiotics started. A chest radiograph is significant for hyperexpansion with bilateral diffuse alveolar and interstitial opacities. He undergoes intubation and receives surfactant twice. Following surfactant administration, he continues to receive mechanical ventilation. He undergoes extubation on day 7 after birth and receives 6 L/min high-flow nasal cannula. He is unable to wean further because of intermittent tachypnea, desaturations, and worsened retractions with weaning. Chest radiography consistently shows diffuse alveolar and interstitial opacities, and the infant has no clinical improvement despite several trials of furosemide, albuterol, budesonide, and fluid restriction (Fig 1). By day 23 after birth, a 3-day course of dexamethasone is tried, which leads to some improvement, allowing the infant to …