CTICasesTracheobronchomegaly with Central Bronchiectasis in Alpha-1 Antitrypsin Deficiency
Imaging findings
A patient with obstructive lung disease and alpha-1 antitrypsin deficiency has a mildly dilated trachea with a corrugated (Mounier-Kuhn-like) contour, weakened cartilage, and redundant floppy posterior membrane that bows outward, along with central bronchiectasis and relatively underwhelming, generalized emphysema. The tracheal diameter measures at the upper limit of normal but with a clearly abnormal, non-round shape.
Key takeaways
Central bronchiectasis with a corrugated, floppy tracheobronchomegaly appearance in an alpha-1 antitrypsin deficiency patient raises the question of whether the airway disease is primary tracheobronchomegaly (Mounier-Kuhn) or a manifestation of alpha-1, which can present predominantly as bronchiectasis. Tracheobronchomegaly is often the end result of various insults to the airway wall, so in an older patient the descriptive term tracheobronchomegaly is preferred over Mounier-Kuhn, which is best reserved for young patients with pure central airway dilation, and the airway morphology matters more than the absolute caliber.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Uncorrected Intracardiac Total Anomalous Pulmonary Venous Return with Eisenmenger PhysiologyCongenital
- Ponytail Artifact Mimicking a Hilar NoduleArtifact
- Granulomatous (Sarcoid-Associated) Pulmonary Veno-Occlusive DiseaseVascular
- Aneurysm of the Membranous Interventricular SeptumCongenital
- Cardiac LipomaNeoplastic
- Posterior Mediastinal LipomaNeoplastic
- High-Grade Aortic Coarctation (Near-Atresia)Congenital
- Recurrent Sternal DehiscenceIatrogenic
See all cases from January 7, 2021 →
Related Congenital cases
- Unroofed Coronary Sinus Variant with Pulmonary Vein Communication
- Pulmonary Artery Sling with Tracheal Anomalies
- Aortic Arch Origin of the Right Vertebral Artery
- Infected Congenital Pulmonary Airway Malformation with Nontuberculous Mycobacteria
- Tracheal Bronchus with Hypoplastic Accessory Lobe
- Pulmonary Atresia with Intact Ventricular Septum