Wang G X, Zhang F Z, Zhao J, Li H B, Duan Q C, Zhang J, Li C F, Ni X, Wang H
Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, National Center for Children's Health(NCCH), Beijing100045, China.
Department of Rheumatology, Beijing Children's Hospital, Capital Medical University, Beijing100045, China.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2024 Nov 7;59(11):1210-1215. doi: 10.3760/cma.j.cn115330-20240326-00173.
To investigate the pathogenesis, clinical signs and diagnosing procedures of relapsing polychondritis(RP) in children with airway involvement. The medical history, clinical symptoms, physical examination, electronic laryngoscopy and imaging findings of six patients were retrospectively analyzed. The patients diagnosed as relapsing polychondritis with involving the airway from January 2018 to December 2021 were in our hospital. The clinical features of the 6 cases were summarized. All 6 patients were male, ranging in age from 8 years 1 month to 14 years 1 month, with a median age of 12.04 years. Stridor and dyspnea were observed in all patients, with hoarseness in 2 patients and frequently nocturnal dyspnea during sleep in 2 patients. Initially, all children were diagnosed as laryngitis or laryngotracheitis, and were treated symptomatically with glucocorticoids and aerosol inhalation. Immunosuppressants and targeted therapy with biologics were given after patients diagnosed as RP. All patients were ultimately required tracheostomy. The time from the onset of airway symptoms to tracheostomy ranged from 1 month to 27 months. Two children had a history of endotracheal intubation prior to tracheostomy. All 6 patients underwent electronic laryngoscopy, revealing involvement of the laryngeal and subglottic mucosa and cartilage structures, which showed gradual improvement with medical therapy. Computed tomography (CT) of the trachea with three-dimensional reconstruction was performed in all patients, demonstrating moderate to severe subglottic stenosis. Two patients exhibited complete airway obstruction at the C4-C6 cervical level. Three children underwent suspension laryngoscopy under general anesthesia and endotracheal mucosal biopsy.Of the 6 children, 3 presented with nasal tip collapse or saddle nose, 2 had auricular cartilage changed, and 1 had scleralinvolvement. One patient underwent PET-CT scanning, which revealed tracheal collapse, diffuse increase in FDG(Flurodeoxyglucose)metabolism with increased FDG uptake in the nasal alar regions. All children were followed up for 2-3 years, 1 child died, while the remaining five continued to receive medical treatment. Relapsing polychondritis with airway involvement has an insidious onset and is difficult to diagnose. The airway stenosisresulting from RP is always severe and necessitating tracheotomy to maintain airway patency in the majority of cases.The treatment coursef or RP is prolonged, requiring long-term tracheostomy tube placement.
探讨儿童复发性多软骨炎（RP）累及气道的发病机制、临床症状及诊断方法。回顾性分析6例患儿的病史、临床症状、体格检查、电子喉镜检查及影像学表现。选取2018年1月至2021年12月在我院诊断为复发性多软骨炎累及气道的患儿。总结6例患儿的临床特征。6例均为男性，年龄8岁1个月至14岁1个月，中位年龄12.04岁。所有患儿均有喘鸣和呼吸困难，2例有声音嘶哑，2例睡眠中常有夜间呼吸困难。最初，所有患儿均被诊断为喉炎或喉气管炎，并给予糖皮质激素及雾化吸入对症治疗。确诊为RP后给予免疫抑制剂及生物制剂靶向治疗。所有患儿最终均需行气管切开术。气道症状出现至气管切开的时间为1个月至27个月。2例患儿在气管切开术前有气管插管史。6例患儿均行电子喉镜检查，显示喉及声门下黏膜及软骨结构受累，经药物治疗后逐渐改善。所有患儿均行气管三维重建CT检查，显示中度至重度声门下狭窄。2例患儿在C4 - C6颈椎水平出现完全气道梗阻。3例患儿在全身麻醉下行支撑喉镜检查及气管黏膜活检。6例患儿中，3例出现鼻尖塌陷或鞍鼻，2例耳廓软骨改变，1例巩膜受累。1例患儿行PET - CT扫描，显示气管塌陷，FDG（氟脱氧葡萄糖）代谢弥漫性增高，鼻翼区域FDG摄取增加。所有患儿随访2 - 3年，1例患儿死亡，其余5例继续接受治疗。复发性多软骨炎累及气道起病隐匿，诊断困难。RP导致的气道狭窄通常严重，多数情况下需要气管切开以维持气道通畅。RP治疗疗程长，需要长期放置气管切开套管。
