A 50-day-old patient, born at term with adequate weight for gestational age and no history of perineum, is taken to the emergency service due to progressive respiratory distress of 12 hours of evolution.
The physical examination revealed severe respiratory distress, universal stripping and apnea episode that reverted with the stimulus.
Chest examination revealed elevated right hemithorax.
Heart rate was 176 beats per minute and respiratory rate was 80 per minute.
An emergency chest X-ray was requested in the intensive care unit and she was admitted to mechanical ventilation for acute imminent respiratory claudication.
Hypotension was observed and crystalloid expansion was initiated up to 60 mL/kg.
Performing lack of response, the patient began to enjoy inotropics/ vasopressors with dopamine and then noradrenaline.
At the same time, a chest X-ray showed a homogeneous radiolucent image in the right lung base, which seemed to correspond to a bullous lesion in the right lower lobe associated with ipsilateral pneumothorax.
Pleural drainage was performed with an oven tube, with improvement of respiratory and hemodynamic parameters.
Once the patient was stabilized, a high-resolution computed tomography of the chest showed a rounded lesion of 6.5 cm in anteroposterior diameter, with air content, septa inside the cavity and pleural nodules hydrorax associated lesion.
An interconsultation with the Surgery Department was carried out, in view of the suspicion of congenital cystic pulmonary disease, which decided to perform surgical resection by right lower lobectomy.
The pathology report of the surgical specimen was cystic adenomatoid type 4.
The patient was admitted favorably and elective extubation was performed.
The patient required transient non-invasive ventilation (CPAP), after which ventilation support and supplementary oxygen were not required.
