Boston Children's Hospital, Harvard Medical School, Division of Pulmonary Medicine, Boston, Massachusetts, USA.
Division of Pediatric Pulmonology, University of Miami Health System, Miami, Florida, USA.
Pediatr Pulmonol. 2024 Jul;59(7):1932-1943. doi: 10.1002/ppul.27015. Epub 2024 Apr 17.
Congenital central hypoventilation syndrome (CCHS) is a rare disease predisposing children to respiratory failure due to abnormal ventilatory drive. Variability in hypoventilation and respiratory support need have been reported. We aim to identify clinical variables associated with incident tracheostomy and common etiologies of hospitalization among children with CCHS.
Hospital discharge records were obtained for children (<21 years) with CCHS hospitalized between 2006 and 2019 from the Kid's Inpatient Database. Primary diagnostic categories for hospitalizations with CCHS were summarized. Multivariable logistic regression models were used to explore risk factors associated with incident tracheostomy.
Among 2404 hospitalizations with CCHS, 133 (5.5%) had incident tracheostomy, 1230 (51.2%) had established tracheostomy, and 1041 (43.3%) had no tracheostomy. Compared with children without tracheostomy, those with incident tracheostomy were younger, had a history of prematurity, congenital heart disease, laryngeal, glottic, and subglottic stenosis (LGSS), congenital airway anomalies, neuromuscular weakness, gastroesophageal reflux disease. Children without tracheostomy had higher mortality than those with tracheostomy status (2.19% vs. 0.66%). Multivariable-adjusted analyses showed that incident tracheostomy was associated with infancy (0-1 years), neuromuscular weakness, and congenital heart disease. Most common diagnostic categories include (1) diseases of the respiratory system (30.23%), (2) injury and poisoning (9.35%), and (3) diseases of the nervous system and sense organs (6.71%).
Children with CCHS who received incident tracheostomy are more likely to be younger and with LGSS, neuromuscular weakness and congenital heart disease. Clinicians should be aware of these risk factors representing more severe CCHS with earlier manifestation needing tracheostomy. Higher mortality among nontracheostomy group highlights the need for considering tracheostomy in caring for children with CCHS.
先天性中枢性肺泡通气不足综合征（CCHS）是一种罕见疾病，由于呼吸驱动异常，儿童易发生呼吸衰竭。已经报道了通气不足和呼吸支持需求的可变性。我们的目的是确定与 CCHS 儿童气管切开术相关的临床变量以及住院的常见病因。
从儿童住院数据库（Kid's Inpatient Database）中获取了 2006 年至 2019 年间患有 CCHS 的<21 岁儿童的住院记录。总结了 CCHS 住院的主要诊断类别。使用多变量逻辑回归模型探讨与气管切开术相关的危险因素。
在 2404 例 CCHS 住院患者中，133 例（5.5%）发生了气管切开术，1230 例（51.2%）有气管切开术，1041 例（43.3%）无气管切开术。与无气管切开术的患儿相比，气管切开术患儿年龄更小，有早产儿、先天性心脏病、喉、声带和杓状软骨狭窄（LGSS）、先天性气道异常、神经肌肉无力、胃食管反流病的病史。无气管切开术患儿的死亡率高于有气管切开术的患儿（2.19%比 0.66%）。多变量调整分析表明，气管切开术与婴儿期（0-1 岁）、神经肌肉无力和先天性心脏病有关。最常见的诊断类别包括（1）呼吸系统疾病（30.23%）、（2）损伤和中毒（9.35%）和（3）神经系统和感觉器官疾病（6.71%）。
接受气管切开术的 CCHS 儿童更有可能年龄更小，且伴有 LGSS、神经肌肉无力和先天性心脏病。临床医生应意识到这些危险因素代表了发病更早、更严重的 CCHS，需要气管切开术。无气管切开术组的死亡率较高，突显了在照顾 CCHS 儿童时考虑气管切开术的必要性。
