A preterm baby boy was born in good condition at 31+5 weeks gestation with a birth weight of 1956 g, following a precipitous labour with no prolonged rupture of membranes and no opportunity for administration of antenatal steroids to mother. Following admission to the neonatal unit, he developed respiratory distress and was commenced on nasal continuous positive airway pressure (CPAP) of 6 cm of water. At 24 hours of age, he developed a left-sided tension pneumothorax (figure 1), requiring endotracheal intubation and insertion of a chest drain. He received two doses of surfactant and was extubated onto CPAP on day 3. There was reaccumulation of the pneumothorax on day 4, which was subsequently drained. He remained self-ventilating in air in the second week of life. From day 15 to day 30, he required humidified high flow nasal cannula oxygen (fractional inspired oxygen up to 0.4), in view of marked subcostal and intercostal recession, intolerance to handling and a compensated respiratory acidosis on capillary blood gases. Figure 2 is the chest radiograph undertaken in the third week of life.
What is the most likely diagnosis in this case?
Congenital pulmonary airway malformation (CPAM)
Respiratory distress syndrome
Pulmonary interstitial emphysema (PIE)
Congenital diaphragmatic hernia
Which of the following is not an effective option for treatment of this condition?
Lateral decubitus with affected side down
High frequency oscillatory ventilation (HFOV)
Selective main bronchial intubation of contralateral lung (which is not affected)
Which of the following statements is false about this condition?
Complications can include other air leak syndromes
Most neonates presenting with this condition have been mechanically ventilated
Diagnosis is usually made on a chest radiograph
Surfactant therapy has been associated with an increase in this condition
Air embolism can be a fatal complication
Questions Answers can be found on page 2
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Contributors SR identified this patient, drafted, reviewed and approved the final manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Parental/guardian consent obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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