Congenital bronchial atresia in infants and children.
OBJECTIVE
Congenital bronchial atresia (CBA) usually presents incidentally in asymptomatic young male adults but is rarely diagnosed in children. The aim of this study was to clarify the clinical characteristics of CBA in childhood and to describe the spectrum of this condition.


METHODS
The clinical features in 29 patients with CBA, aged from 1 day to 13 years (median, 4 years), were reviewed retrospectively. Diagnosis was confirmed by pathological findings of a blind-ending bronchus associated with distal mucous-filled bronchocele surrounded by hyperinflated lung parenchyma.


RESULTS
All but 1 patient were symptomatic. The most frequent symptom was productive cough and fever owing to recurrent pneumonia found in 26 children. Two infants suffered from respiratory distress. Chest x-ray showed various findings of infiltrative pneumonia, emphysema, and a large cyst. Computed tomography, bronchography, and bronchoscopy were useful modalities for demonstrating bronchocele associated with hyperinflated lung or proximal blind-ending bronchus even in infected cases. The right lower lobe was predominantly affected in 12 cases, followed by left or right upper lobe in 7 cases. Lobectomy or segmentectomy resulted in remarkable clinical improvement.


CONCLUSIONS
Congenital bronchial atresia presents differently in children than in young adults. Modern imaging techniques and careful pathological analyses lead to an accurate diagnosis of bronchial atresia, which may be misdiagnosed as intralobar sequestration or pulmonary bronchial cysts. Bronchial atresia is a distinct pathological entity that accounts for recurrent pneumonia or respiratory distress in childhood, requiring surgical treatment.
Morikawa
Nobuyuki
N
Division of General Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan. morikawa-n@ncchd.go.jp
Kuroda
Tatsuo
T
Honna
Toshiro
T
Kitano
Yoshihiro
Y
Fuchimoto
Yasushi
Y
Terawaki
Kan
K
Kawasaki
Kazuteru
K
Koinuma
Goro
G
Matsuoka
Kentaro
K
Saeki
Morihiro
M
D000293:Adolescent; D001994:Bronchogenic Cyst / Q000175:diagnosis*; D001999:Bronchoscopy; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011013:Pneumonectomy / Q000379:methods*; D011014:Pneumonia / Q000209:etiology; D018633:Pulmonary Atresia / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery*; D013902:Radiography, Thoracic; D012008:Recurrence; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome