Long Term Ventilation in Pediatric Central Apnea: Etiologies and Therapeutic Approach over a Decade.

Abstract

This retrospective study aimed to analyze the clinical characteristics, ventilatory strategies, and effectiveness of ventilation in pediatric patients with central apneas treated at the Sleep Medicine and Long-Term Ventilation Unit of the Bambino Gesù Children's Hospital in Rome from 2012 to 2022. Among all ventilated patients at our Center from January 2012 to December 2022, we retrospectively included children with a cAHI ≥ 1 events/h on baseline poly(somno)graphic study. Additional parameters assessed included the underlying disease, type of ventilation (non-invasive vs. invasive), age at ventilation onset, ventilation mode, and transcutaneous capnometry parameters. To assess the effectiveness of ventilation on central apneas, we compared the cAHI at baseline and on ventilation. Sixty-seven patients met the inclusion criteria for central apnea (cAHI > 1 events/h). Diagnoses included hypoxic-ischemic encephalopathy, 15 (22.4%); Ondine syndrome, 14 (20.9%); polymalformative syndrome, 10 (14.9%); Prader-Willi syndrome, 8 (11.9%); brain tumor, 6 (9.0%); Down syndrome, 4 (6.0%); ROHHAD syndrome, 2 (3.0%); other infrequent pathologies were, Arnold-Chiari II, primary central apnea, epilepsy, lisosomal diseases, hydrocephalus, myopathy, obesity, Rett Syndrome. Pressure-supported ventilation (PSV) was the most common mode used (45 out 67 patients, 67.2%), followed by pressure-controlled ventilation (PCV) (15 out 67 patients, 22.4%) and continuous positive airway pressure (CPAP) (7 out 67 patients, 10.4%). Statistically significant improvement (p < 0.05) in cAHI was observed in patients with polymalformative syndrome (3.5 vs. 0.3, p = 0.01), hypoxic-ischemic encephalopathy (3.1 vs. 0.1, p = < 0.01), and Prader-Willi syndrome (3.5 vs. 0.1, p = 0.03), while there was no significant improvementn in children with brain tumor (6.2 vs. 1.5, p = 0.21). Central apneas are present in children with various underlying pathologies. Ventilatory strategies tailored to the specific diagnosis and severity of central apneas yield significant improvements in cAHI. PSV was the preferred ventilation mode in this study and there was notable effectiveness across different diagnostic categories. PCV was employed in most severe cases. CPAP was exclusively used in patients with predominantly obstructive sleep apneas.