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Unilateral pulmonary agenesis presenting as an airway lesion
B L Bentsianov1, N A Goldstein, R Giuste
1Department of Otolaryngology, 134 Atlantic Ave, Brooklyn, NY 11201, USA.
Insights
Unilateral pulmonary agenesis can mimic common airway issues in children, leading to misdiagnosis. Early recognition on chest radiographs is crucial to avoid unnecessary bronchoscopy and ensure correct treatment for this rare condition.
Area of Science:
- Pediatric Pulmonology
- Diagnostic Imaging
- Thoracic Surgery
Background:
- Otolaryngologists often perform rigid bronchoscopy for suspected foreign body aspiration or bronchial lesions in children.
- Chest radiographs showing unilateral lung collapse with mediastinal shift commonly prompt bronchoscopy referrals.
Observation:
- This study presents two pediatric cases with unilateral pulmonary agenesis and aplasia exhibiting typical radiologic findings suggestive of airway obstruction.
- Diagnosis was confirmed during bronchoscopy, highlighting a potential delay in preoperative suspicion.
Findings:
- Unilateral pulmonary agenesis can present with radiologic signs mimicking common pediatric airway lesions.
- Preoperative suspicion may be enhanced by reviewing chest radiographs for chronic contralateral chest wall changes and lung expansion.
Implications:
- Misdiagnosing pulmonary agenesis can lead to unnecessary bronchoscopy risks, including potential bronchial perforation.
- Considering unilateral pulmonary agenesis in the differential diagnosis of pediatric airway lesions is essential for accurate and timely management.
Abstract:
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. Arch Otolaryngol Head Neck Surg. 2000;126:1386-1389