CTICasesNeurofibromatosis-Related Cystic Lung Disease and Emphysema
Imaging findings
CT demonstrated severe emphysema, particularly in the upper lobes with some central dot signs, but also revealed well-defined thin-walled cysts that were clearly distinct from emphysema, especially in the lower lung zones. Cutaneous neurofibromas were also visible on the soft tissue windows.
Key takeaways
Neurofibromatosis can cause a combination of emphysema (often smoking-related) and thin-walled cystic lung disease. Recognition of both features, along with cutaneous neurofibromas, can lead to the diagnosis of neurofibromatosis-related lung disease.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Incidental Pulmonary Embolism on Coronary CTAVascular
- Mycobacterium Avium Complex (MAC) Lung Nodule Mimicking MalignancyInfection
- Presumed Takayasu Arteritis (Extensive Aortic Vasculitis)Autoimmune
- Metastatic Squamous Cell Carcinoma of the Skin Obscuring Anterior MediastinumNeoplastic
- Lung Adenocarcinoma Obscured by Pacemaker GeneratorNeoplastic
- Iatrogenic Arterial Line Placement into Right Subclavian Artery and AortaIatrogenic
- Cryptococcal Endobronchial Mass with Post-Obstructive PneumoniaInfection
- Neurofibromatosis Type 1 (NF1) with Extensive Cystic Lung Disease and EmphysemaCongenital
See all cases from October 14, 2016 →
Related Congenital cases
- Bicuspid Aortic Valve with Aortopathy
- Pentalogy of Fallot
- Coarctation of the Aorta with Classic '3 Sign'
- Pulmonic Valvular Stenosis with Post-Stenotic Dilation of the Main and Left Pulmonary Arteries
- Anomalous Left Superior Pulmonary Vein Drainage (Variant Anatomy)
- Incidental Systemic Arterial Supply to the Right Lower Lung