Congenital bronchopulmonary foregut malformation: systematic review of the literature

BMC Pediatr. 2019 Sep 2;19(1):305. doi: 10.1186/s12887-019-1686-1.


Background: Congenital bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory system. Early diagnosis is difficult, and delayed recognition may result in considerable complications. The aim of the study was to identify and analyze the clinical characteristics and radiological features of CBPFMs.

Methods: A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Ovid database, EMBASE were searched for relevant publications to identify all published case-reports of CBPFM since 1992. Data about the demography, clinical presentation, pathology, imaging features, treatment and prognosis were collected.

Results: Sixty-one cases were included in our study. Cases were aged from 1 day to 59 years with the majority aged 3 years or younger. The most common type was group III (37.7%), followed by group II (29.5%)group I (27.9%) and group IV (4.9%). The presentations included respiratory distress (32.8%), cough/choking following food intake and other presentations associated respiratory infection. Thirty-eight cases (62.3%) were diagnosed by upper gastrointestinal series (UGI). Misdiagnosis was common. Eight cases (13.1%) of the included cases died.

Conclusions: Early recognition and extensive delineation of the anatomy of CBPFM are important to correct these anomalies successfully. UGI is the first choice to confirm the abnormal bronchus communicating with the esophagus. Resection of abnormal pulmonary tissue, lobe or even unilateral lung is preferred. Reconstruction procedures are feasible in selected patients.

Keywords: Congenital bronchopulmonary foregut malformation; Esophageal bronchus; Esophageal lung; Systematic review.

Publication types

  • Case Reports
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Bronchi / abnormalities*
  • Bronchi / diagnostic imaging
  • Child
  • Child, Preschool
  • Dextrocardia / diagnostic imaging
  • Esophageal Atresia / complications*
  • Esophageal Atresia / diagnostic imaging
  • Esophageal Atresia / surgery
  • Esophagus / abnormalities*
  • Esophagus / diagnostic imaging
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Lung / abnormalities
  • Lung / diagnostic imaging
  • Male
  • Middle Aged
  • Pulmonary Artery / abnormalities
  • Pulmonary Artery / diagnostic imaging
  • Stomach / abnormalities*
  • Stomach / diagnostic imaging
  • Tracheoesophageal Fistula / complications*
  • Tracheoesophageal Fistula / diagnostic imaging
  • Tracheoesophageal Fistula / surgery
  • Young Adult

Supplementary concepts

  • Esophageal atresia with or without tracheoesophageal fistula