A rare cause of respiratory distress in preterm infants: a case report of acquired subglottic cysts

Born at 25 weeks + 2 days gestational age by emergency section due to unstoppable labour with rupture of the membranes in a monochorionic diamniotic twin pregnancy. Pregnancy was characterised by a twin-to-twin transfusion syndrome (receving fetus). Incomplete lung maturity. At birth, there was no respiratory activity and the heart rate (HR) was below 100 bpm. Need for intubation at 4 min of life due to suboptimal oxigen saturation. The first dose of endotracheal surfactant was administered due to the persistent suboptimal saturations and HR < 100 bpm with FiO2 at 100 at 10 min of life. This resulted in a gradual improvement ofvital parameters. The patient was transferred to the Neonatal Intensive Care Unit (NICU) with mechanical invasive respiratory support. During hospitalization, the infant was supported by invasive mechanical ventilation for 18 days. Two doses of surfactant were administered at 24 h and 96 h of life. The patient was discharged at 40 weeks post-menstrual in good general condition, with a mild form of bronchopulmonary dysplasia (BPD) (grade 1 according to Jansen et al. [5]). He was readmitted to hospital at 43 weeks post-menstrual age due to thedevelopment of mixed stridor (inspiratory-expiratory) and worsening of respiratory symptoms, requiring the initiation high-flow oxygen therapy. Initially, we performed infection screening with blood culture (negative), urinalysis (negative), chest-x-ray and ultrasound (without evidence of anomalies), and multiplex reverse-transcriptase polymerase chain reaction (RT-PCR) on nasopharyngeal swabs for respiratory viruses and bacteria (including Mycoplasma pneumoniae, Chlamydophila pneumoniae, Legionella pneumoniae, and Bordetella pertussis)which were negative. Echocardiography was performed to assess cardiac patterns, and the results were normal. Due to the persistence of stridor, we performed an upper airway evaluation with direct laryngoscopy, which revealed type two laryngomalacia associated with mucosal oedema. After this evaluation, the patient’s clinical condition progressively worsened, with persistent stridor and an increased need for ventilatory support. In consultation with an otolaryngologist (ENT), we decided to perform laryngoplasty surgerythat discovered numerous SGCs which almost completely obstructed the airway (Fig. 1).

Fig. 1figure 1

Laryngoscopy showing glottic and subglottic cysts with narrowing of the airway patency

The ENT decided to remove the SGCs bycold excision method and performed a tracheostomy. A cuffed cannula of 2.5 mm diameter was positioned. No complications occurred during the positioning. There was a gradual improvement in respiratory condition. Post-surgery laringoscopy showed complete resolution with minimal oedema. (Fig. 2). Histopathological examination confirmed the benign nature of the lesion.

Fig. 2figure 2

Post-operative control under endoscopic evaluation showing a better airway patency although a slight glottic edema persists

Initially, non-invasive ventilation support (CPAP) was placed. The next day, we assisted in the progressive improvement of clinical conditions, with the possibility of withdrawing ventilatory support after 8 days from surgery. Subsequent controls showed a completely patent airway and absence of SGCs. The tracheostomy tube was replaced twice during the hospital stay. The infant was discharged one monthafter surgery, with anti-reflux therapy. The tracheostomy was closed and left in placeSubsequent checks showed the presence of fibrous material associated with micro-cysts (maximum diameter 0.4 millimeters). Therefore, as a precaution, the endotracheal tube was not removed. 40 days after discharge, the tube is permanently removed.

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