儿童气管幼年性黄色肉芽肿1例报告并文献复习

Juvenile xanthogranuloma presenting as a solitary tracheal mass in a child:a case report and literature review

目的探讨儿童气管幼年性黄色肉芽肿的临床特征和治疗方法。方法回顾分析1例气管幼年黄色肉芽肿患儿的临床资料并复习相关文献。结果患儿,女,1岁10个月,以反复喘息为主要表现,常规抗感染、全身糖皮质激素及支气管舒张剂治疗效果不佳。电子支气管镜提示患儿声门下气管侧壁可见1个直径约0.8 cm的淡黄色肿物,表面光滑,阻塞气管腔约60%。经显微喉镜下激光切除肿物,手术局部行CO2冷冻治疗,病理及免疫组化提示为幼年性黄色肉芽肿。随访5个月未见复发。检索建库至2020年8月中国知网中文数据库、万方数据库及PubMed,以"幼年性黄色肉芽肿"和"儿童"为关键词组合检索,共检索到8篇文献报道了8例儿童气管或喉部幼年性黄色肉芽肿者。结论儿童气管幼年性黄色肉芽肿罕见,当出现严重气道梗阻时,可采取手术切除治疗。激光切除肿物联合CO2冷冻切口,既可解除气道梗阻,又能减少局部肉芽形成。

Objective To investigate the clinical characteristics and treatment of juvenile xanthogranuloma (JXG) presenting as a solitary tracheal mass in children.Methods Clinical data of a patient admitted to the first affiliated hospital of Xiamen University with tracheal JXG in March 2019 was retrospectively analyzed,and related literatures were reviewed.Results A 20-month-old girl,had a course of more than 2 months with repeated stridor and inspiratory dyspnea.She was treated with inhalation budesonide and alternating albuterol in addition to a systemic steroid,but relief was minimal.Evaluation of the subglottic area by bronchoscopy identified a light-yellow mass with a diameter of about 0.8 cm on the lateral wall of the trachea below the glottis which resulted in 60% of the trachea obstructed.The tumor was removed by laser under the microlaryngoscope,and locally treated with CO2 cryotherapy.Histopathological and immunohistochemical examination confirmed the diagnosis of JXG involvement of the trachea.Follow up for 2 months showed no recurrence.With the "juvenile xanthogranuloma,children" as keywords,the CNKI,Wangfang digital knowledge service platform and PubMed were searched for relevant articles from the establishment to August 2020.Eight cases of children with JXG of trachea or larynx were reported.Including this case,all the 9 patients had recurrent wheezing as the main manifestation,and the conventional anti-infection and antiasthmatic treatment did not have a good effect.Some of the cases were surgically excised,some were conservatively treated after tracheotomy,waiting for the natural degeneration of the lesion,and most of them had a good prognosis.Conclusion JXG of trachea or larynx is rarely seen in children.JXG can remit spontaneously in some cases,therefore surgical treatment was only required when severe airway obstruction occurs.Laser resection of tumor combined with CO2 cryosurgery can not only relieve airway obstruction,but also reduce the formation of local granulation tissue.