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Clinical Note
November 2000

Unilateral Pulmonary Agenesis Presenting as an Airway Lesion

Author Affiliations

From the Departments of Otolaryngology (Drs Bentsianov, Goldstein, and Har-El) and Pediatrics, (Dr Giuste), Long Island College Hospital, Brooklyn, NY; and the Department of Otolaryngology, State University of New York Health Science Center at Brooklyn (Drs Bentsianov, Goldstein, and Har-El).

Arch Otolaryngol Head Neck Surg. 2000;126(11):1386-1389. doi:10.1001/archotol.126.11.1386

Otolaryngologists are frequently consulted to perform rigid bronchosopy in children with suspected foreign body aspiration, mucous plug occlusion of a mainstem or lobar bronchus, or other bronchial mass lesions. Chest radiographs that demonstrate unilateral lung or lobar collapse with a shift of mediastinal structures toward the affected side often prompt this referral. We describe 2 children, one with unilateral pulmonary agenesis and one with pulmonary aplasia, who presented with these radiologic findings. In each case, the definitive diagnosis was made at the time of bronchosopy. The diagnosis might have been suspected preoperatively if the chest radiographs had been reviewed with this clinical entity in mind. Because of its variable clinical presentation, diagnosis requires a high index of suspicion. Although computed tomography of the chest is diagnostic, the diagnosis may be suggested by chronic changes in the contralateral aspect of the chest wall and lung expansion on chest radiographs. Misdiagnosis may subject the patient to the unnecessary risks of bronchoscopy and to potential perforation of the rudimentary bronchus. Although pulmonary agenesis is a rare entity, it may mimic more common airway lesions. Therefore, unilateral pulmonary agenesis should be considered in the differential diagnosis of pediatric airway lesions.