Objective: To investigate the clinical characteristics, treatment and prognosis of congenital pulmonary airway malformation (CPAM) complicated with chronic pulmonary aspergillosis (CPA) in children. Methods: The clinical manifestation, laboratory test, radiological feature, treatment and prognosis of 4 pediatric patients diagnosis of CPAM with CPA ascertained between March 2016 and April 2020 at the Department of No.2 Respiratory Medicine and Thoracic Surgery of Beijing Children's Hospital were retrospectively analyzed. Results: The 4 children included 2 males and 2 females, their age ranged from 9.9 to 13.6 years. Cough presented in 3 cases, hemoptysis in 2 cases, whereas in 1 case, pneumothorax was the first manifestation of the condition. Past history revealed multiple hospital admissions for pneumonia in 2 children and 1 with more than 2 episodes of wheezing. Aspergillus IgG was positive in 3 patients. Significantly elevated total IgE and fumigatus-specific IgE levels were noted in 2 children. CT demonstrated multiple cystic lesions, and fungal balls were seen in 2 children. They all underwent lobectomy and the lung tissue from the 4 children all demonstrated CPAM with Aspergillus infection. Aspergillus fumigatus was isolated from the abscess collected during operation in 1 patient. Postoperative, voriconazole was given in 3 children for 8 weeks, recurrence was observed in 2 children with characteristics of allergic bronchopulmonary aspergillosis. Voriconazole was given in one patient for 12 weeks with no recurrence. Conclusions: CPAM might be complicated with CPA,if there are hemoptysis, and fungal ball in chest CT, cautious preoperative examinations for microorganism are necessary. The necessity and duration of postoperative antifungal therapy need to be determined on individual basis.
目的： 探讨先天性肺气道畸形（CPAM）合并慢性肺曲霉病（CPA）患儿的临床特点及治疗转归。 方法： 回顾性分析2016年3月至2020年4月在北京儿童医院呼吸二科及胸外科住院确诊的4例CPAM合并CPA患儿的临床表现、实验室检查、影像学特点、治疗及预后。 结果： 4例CPAM合并CPA患儿中男2例、女2例，年龄9.9~13.6岁。慢性咳嗽3例，咯血2例，1例以气胸为首发表现；3例为过敏体质，2例有反复肺炎史，1例有喘息史。3例血曲霉菌IgG阳性，1例未查，2例血总IgE及烟曲霉特异性IgE显著升高；4例肺CT均表现为肺内囊性病变，2例见曲霉球；4例均行肺叶切除术，病理结果均示CPAM合并曲霉菌感染，1例术中脓液培养示烟曲霉。3例术后予伏立康唑治疗8周，2例复发且在围手术期均呈高度过敏状态，血总IgE及烟曲霉特异性IgE显著升高，其中1例复发后呈变应性支气管肺曲霉菌病表现；另外1例术后伏立康唑治疗12周无复发。 结论： CPAM可能为CPA的危险因素，患儿出现咯血和（或）肺CT可见曲霉球具有提示意义，术前应积极行病原学检查。术后是否需要抗真菌治疗及疗程尚无统一建议。.