Vertically fused tracheal cartilage. An underrecognized anomaly.

ABSTRACT

Anterior vertical fusion of the tracheal cartilages with normal pars membranacea is a rarely described tracheal anomaly. We report four cases of this anomaly, three associated with craniosynostosis syndromes and one with Goldenhar's syndrome. The anomaly was documented at postmortem examination in one case, both endoscopically and at tracheotomy in two cases, and endoscopically in one case. Documentation from gross postmortem photographs, photomicrographs, and intraoperative endoscopic photographs is presented so that the anatomical aspects of this anomaly can be thoroughly understood and recognized. All four newborn patients developed recurrent lower respiratory tract infections, reactive airway disease, and chronically retained secretions attributable to this anomaly. Neither tracheal stenosis nor stridor was encountered. In the three surviving patients, the infections grew less frequent and less severe in later childhood. These observations suggest that this tracheobronchial anomaly may be associated with a variety of craniofacial syndromes and may alter airflow dynamics in a way that predisposes to retained secretions and chronic pulmonary disease.