Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Shaun M Kunisaki, Dario O Fauza, Luanne P Nemes, Carol E Barnewolt, Judy A Estroff, Harry P Kozakewich, Russell W Jennings
Journal of Pediatric Surgery 2006, 41 (1): 61-5; discussion 61-5

PURPOSE: This study was aimed at determining whether different congenital lung masses represent diverse manifestations of a single developmental abnormality associated with fetal airway obstruction.

METHODS: We conducted a 3-year retrospective review of patients who underwent surgical resection of a prenatally diagnosed lung mass. Prenatal imaging was used to define mass position and its effect on adjacent organs. Lung specimens were examined through careful full-specimen microdissections, as well as by plain and contrast roentgenograms.

RESULTS: Twenty-five patients underwent lung resection during this study period. Based on the final pathology reports, 56% were congenital cystic adenomatoid malformations, 12% were congenital lobar emphysemas, 8% were bronchopulmonary sequestrations, and 24% had features of both cystic adenomatoid malformation and bronchopulmonary sequestrations. No bronchogenic cysts were present in this series. Overall, bronchial atresia was identified in 77% of the examined specimens (n = 22) and was associated with all types of lung malformations.

CONCLUSIONS: Bronchial atresia is a common, unrecognized component of prenatally diagnosed congenital cystic adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysemas, and lesions of mixed pathology. Most congenital lung masses may be part of a spectrum of anomalies linked to obstruction of the developing fetal airway as an underlying component in their pathogenesis.

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