By definition, this is the group of malformations of the embryonic foregut characterized by persistent communication between the esophagus or stomach and part of the respiratory system.

There are 4 subgroups:

-        I: associated with esophageal atresia with or without esotracheal fistula,

     *        Ia: the entire lung is connected to the bronchus originating from the esophagus or stomach.

     *        Ib: part of the lung is connected to the bronchus originating from the esophagus or stomach

-        II:   the entire lung is connected to the bronchus originating from the esophagus

-        III: part of the lung (extralobar sequestration) is connected to the bronchus originating
       from the esophagus or stomach; vascularization via the aorta or a hypoplastic branch
        of the pulmonary artery.

-        IV:        part of the normal bronchial tree (right (43 %) or left (22 %) lower lobe)
       communicates with the esophagus (lower 2/3); the part of the lung
       from which the bronchus originates has systemic vascularization.

Including:

• esotracheal fistulas
• laryngeal cleft (especially III and IV)
• congenital pulmonary malformations (formerly: adenomatoid disease of the lung)
• intra- and extralobar sequestrations
• esophageal bronchus
• bridging bronchus
• bronchoesophageal fistulas
• esophageal or gastric duplications
• congenital tracheal atresia or stenosis
• tracheal diverticula
• esophageal trachea
• congenital lobar emphysema

and frequently associated:

-        VACTERL syndrome (10 %)

-        cardiac malformation (18 %)

-        vertebral malformation (10 %)

-        anorectal malformation (3 %)

-        diaphragmatic hernia (3 %)

Anesthetic implications: 

according to the precise type of malformation (see specific items)

References : 

-        Srikanth MS, Ford EG, Phillip S, Mahour GH.
Communicating bronchopulmonary foregut malformations: classification and embryogenesis.
J Pediatr Surg 1992; 27: 732-6.

-        Désir A, Ghaye B.
Congenital abnormalities of the intrathoracic airways.
Radiol Clin N Am 2009; 47: 203–25  

-        Yang G, Chen L, Xu C,Yuan M, Li Y.
Congenital bronchopulmonary foregut malformation: systematic review of the literature.
BMC Pediatrics 2019 ; 19:305  doi.org/10.1186/s12887-019-1686-1

-        Tambucci R, Wautelet O, Haenecour A, François G, Goubeau C, Scheers I etc.
Esophageal trachea, a unique foregut malformationn requiring multistage reconstruction : case report.
Front Pediatr 2020 ; 8 : 605143.

Updated: October 2024