Presti Santiago, Pavone Martino, Verrillo Elisabetta, Paglietti Maria Giovanna, Del Colle Anna, Leonardi Salvatore, Cutrera Renato
Pediatric Pulmonology and Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Academic Department of Pediatrics, Pediatric Hospital "Bambino Gesù" Research Institute, Rome, Italy.
Pediatric Respiratory and Cystic Fibrosis Unit, Department of Clinical and Experimental Medicine, "San Marco" Hospital, University of Catania, Catania, Italy.
Pediatr Pulmonol. 2025 Jan;60(1):e27400. doi: 10.1002/ppul.27400. Epub 2024 Nov 18.
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.
本回顾性研究旨在分析2012年至2022年在罗马的 Bambino Gesù 儿童医院睡眠医学与长期通气科接受治疗的中枢性呼吸暂停小儿患者的临床特征、通气策略及通气效果。
在2012年1月至2022年12月期间在我们中心接受通气治疗的所有患者中，我们回顾性纳入了在基线多导睡眠图研究中cAHI≥1次/小时的儿童。评估的其他参数包括潜在疾病、通气类型（无创通气与有创通气）、通气开始时的年龄、通气模式和经皮二氧化碳监测参数。为评估通气对中枢性呼吸暂停的效果，我们比较了基线时和通气时的cAHI。
67例患者符合中枢性呼吸暂停的纳入标准（cAHI>1次/小时）。诊断包括缺氧缺血性脑病15例（22.4%）；翁丁氏综合征14例（20.9%）；多发畸形综合征10例（14.9%）；普拉德-威利综合征8例（11.9%）；脑肿瘤6例（9.0%）；唐氏综合征4例（6.0%）；ROHHAD综合征2例（3.0%）；其他罕见疾病包括阿诺德-奇亚里II型、原发性中枢性呼吸暂停、癫痫、溶酶体病、脑积水、肌病、肥胖、雷特综合征。压力支持通气（PSV）是最常用的模式（67例患者中有45例，67.2%），其次是压力控制通气（PCV）（67例患者中有15例，22.4%）和持续气道正压通气（CPAP）（67例患者中有7例，10.4%）。多发畸形综合征患者（3.5对0.3，p = 0.01）、缺氧缺血性脑病患者（3.1对0.1，p = <0.01）和普拉德-威利综合征患者（3.5对0.1，p = 0.03）的cAHI有统计学意义的改善（p < 0.05），而脑肿瘤患儿（6.2对1.5，p = 0.21）没有显著改善。
中枢性呼吸暂停存在于患有各种潜在疾病的儿童中。根据中枢性呼吸暂停的具体诊断和严重程度量身定制的通气策略可使cAHI显著改善。PSV是本研究中首选的通气模式，在不同诊断类别中均有显著效果。PCV用于大多数严重病例。CPAP仅用于以阻塞性睡眠呼吸暂停为主的患者。
