Congenital tracheal stenosis complicated with communicating bronchopulmonary foregut malformation in 2 children and literature review / 中华实用儿科临床杂志

Objective:To explore the clinical typing, diagnostic method and treatment plan of congenital tracheal stenosis (CTS) combined with communicating bronchopulmonary foregut malformation (CBPFM) in children.Methods:The clinical data of 2 children with CTS and CBPFM who were treated in the Center for Respiratory Intervention of Children′s Hospital Affiliated to Shandong University in May 2021 and January 2022 were retrospectively analyzed.Studies were retrieved from domestic and foreign databases, so as to summarize the clinical characteristics of CTS complicated with CBPFM and investigate the typing method of CBPFM.Results:One patient was a 4-year-old girl, who sought the medical advice due to " recurrent cough and asthma for more than 4 years" . She was diagnosed with typeⅡ CBPFM at the right side and CTS.Surgical thoracoscopic right pneumonectomy plus oesophageal repair was performed.The other patient was a 7-month-and-2-day-old female, who visited the hospital for " difficult eating, dyspnea and purple lip cyanosis for 7 months" . This patient was diagnosed with typeⅡ CBPFM at the left side and CTS.Slide tracheoplasty and left pneumonectomy+ oesophageal repair were performed successively.Eight English and one Chinese studies were collected.Twenty-one children with CBPFM and 12 children with CTS and CBPFM were included.Eleven CTS cases with sufficient diagnostic evidence were complicated with typeⅠA and typeⅡCBPFM.Conclusions:CTS and CBPFM can lead to severe wheezing and dyspnea.Clinicians should enhance their awareness and be more cautious.There may be a potential link between CTS and typeⅠA and typeⅡCBPFM, and further investigation is required.

Bronchopulmonary foregut malformation mimicking pseudocyst of pancreas.

We report a four-yr-old girl who was successfully treated for a large gastro-duodenal duplication that communicated with extra-lobar pulmonary sequestration on one end and the main pancreatic duct on the other. Such an association has not been reported hitherto.

Congenital Bronchopulmonary Foregut Malformation: Analysis of the surgical and autopsy cases

Because early embryonic development of the tracheobronchial tree and foregut are closely associated, there is a wide spectrum of congenital anomalies involving either one or both organ systems. We analysed a total of 89 surgical and autopsy cases that are assumed to belong to congenital bronchopulmonary foregut malformation from the files of Seoul National University Hospital and Children's Hospital during the periord of 1961~1990. We also reviewed the serial sections of the embryos and fetuses from 3 weeks to fifteen weeks fertilization age for the observation of tracheobronchial and esophageal trees. Intralobar sequestrations(25 cases) and extralobar pulmonary sequestrations(4 cases) with patent, involuted-partial or complete-communication with the alimentary tract, tracheoesophageal fistula(30 cases) with or without esophageal atresia, esophageal atresia, esophageal stenosis due to tracheobroncheal remnant(4 cases), foregut duplication cysts(3 cases), esophageal or gastric diverticulum(1 cases), and bronchogenic cysts(22 cases) are included in this analysis(Table 1). Through this study, we confirmed the unifying concept of "bronchopulmonary forgut malformations". We believe a common embryologic pathogenesis leads to the formation of a previously described spectrum of malformations.