[Clinical characteristics of bronchopulmonary foregut malformation]

Zhonghua Er Ke Za Zhi. 2017 Aug 2;55(8):628-631. doi: 10.3760/cma.j.issn.0578-1310.2017.08.016.
[Article in Chinese]

Abstract

Objective: To analyze the clinical characteristics, diagnosis and treatment of bronchopulmonary foregut malformation(BPFM). Method: The clinical manifestations, imaging findings and treatment of 8 patients with BPFM were analyzed retrospectively from January 2006 to May 2016 in Beijing Children's Hospital. Result: The age of children varied from 2 months to 7 years and 3 children were male while 5 female. Symptoms showed cough in 6 cases, fever in 4 cases, bucking when intaking of fluids or foods in 3 cases, tachypnea in 1 case, wheezing in 1 case, vomiting in 1 case, haematemesis in 1 case Pulmonary signs were decreased breath sounds in 4 cases, phlegm rale in 3 cases, shortness of breath in 2 cases, wheeze in 1 case, and retraction in 1 case. The upper gastrointestinal series showed abnormal fistulous tracts arising from the esophagus or the gastric fundus and extending into the mass in the lung. CT showed pulmonary sequestration and prompted the tube between lung and esophagus. Six children underwent pneumonectomy and esophageal fistula repair. They were discharged and their symptoms were improved. Two cases of children were discharged from a hospital without surgery. Conclusion: Bronchopulmonary foregut malformation usually has its onset in early stage of life. The most common symptoms include recurrent pneumonia or bucking when intaking of fluids or foods. CT can demonstrate the bronchopulmonary sequestration and evaluate the communication with the gastrointestinal tract. The upper gastrointestinal series can demonstrate the abnormal tract directly. Pneumonectomy and esophageal fistula repair are the treatment of this disease.

目的: 分析儿童支气管肺前肠畸形临床特征和诊治要点。 方法: 对2006年1月—2016年5月首都医科大学附属北京儿童医院收治的8例支气管肺前肠畸形患儿的临床表现、影像学检查、治疗转归进行回顾性分析。 结果: 8例中男3例,女5例;年龄2月龄~7岁。症状表现:咳嗽6例、发热4例、进食后呛咳3例、气促1例、喘息1例、呕吐1例、呕血1例。肺部体征中肺部呼吸音减低4例、痰鸣音3例、呼吸急促2例、喘鸣音1例、吸气性三凹征1例。上消化道造影显示对比剂于食管下段或胃经异常通道进入隔离肺内。CT检查显示隔离肺组织,提示呼吸道与消化道间有异常通道。6例患儿接受肺切除并食管瘘修补术,术后症状好转出院。2例未接受手术治疗自动出院。 结论: 支气管肺前肠畸形发病年龄小,临床表现为反复呼吸道感染或进食后呛咳。胸部CT可以显示肺隔离症及其与消化道相通情况。上消化道造影可以直接显示出支气管肺前肠畸形的异常通道。外科行肺切除并食管瘘修补术是本病的治疗方法。.

Keywords: Bronchopulmonary sequestration; Diagnosis; Radiography; Signs and symptoms.

MeSH terms

  • Bronchi* / abnormalities
  • Bronchopulmonary Sequestration* / diagnosis
  • Bronchopulmonary Sequestration* / surgery
  • Child
  • Esophageal Fistula* / diagnosis
  • Esophageal Fistula* / surgery
  • Female
  • Humans
  • Male
  • Retrospective Studies