Thanks to Ale for sharing a case of a young man without any significant PMH who had chest pain and cough and abnormal imaging. Sent to ED with left upper lobe pneumothorax that improved with a small bore chest tube.
What’s the differential for spontaneous pneumothorax in this kind of case? What is the long-term management?
https://www.nejm.org/doi/full/10.1056/nejmoa1910775 – RCT of 316 patients randomized to intervention vs conservative management for spontaneous pneumothorax; conservative management was noninferior.
There was significant family history of pneumothoraces. A review of genetic causes of pneumothorax: https://pubmed.ncbi.nlm.nih.gov/30681372/.
A CT showed some cysts – we reviewed the wide range of causes of this:
Key points in the chat during this asked about skin findings as suspicion climbed for Birt Hogg Dube syndrome.
- skin lesions develop in 80% of patients in 30s-40s, fibrofolliuclomas in mid face
- 7x increased risk of malignancy especially renal cancer > annual surveillance with US
- genetic testing (refer to Center for Genetics), counseling, screening of family members (autosomal dominance inheritance)
- consider early ipsilateral VATS pleurodesis after pneumothorax
Bonus: case report by Dr. Kalhan and Dr. Jain
Thanks, Ale!
Alexandra Hanrahan, MD, Pulmonary and Critical Care