CTICasesNiemann-Pick Disease — Additional Cases
Imaging findings
CT from 2002-2005 in an older patient who had prior cardiac surgery demonstrates septal thickening throughout both lungs without pleural effusion. The pattern resembles pulmonary edema or fibrosis at first glance but the absence of pleural effusion and normal heart size are important negative findings. An older scan from 2002 shows the same pattern unchanged prior to cardiac surgery, excluding pulmonary venous hypertension as the cause. Air trapping is also noted on some images, potentially related to old histoplasma granulomas obstructing small airways. Pathologic confirmation confirms Niemann-Pick disease.
Key takeaways
Niemann-Pick disease type B may be encountered incidentally on chest CT obtained for non-respiratory indications (such as pre-operative cardiac evaluation), where the finding of unexplained diffuse septal thickening without pleural effusion and with a normal heart size should prompt consideration of storage disease. The unchanged appearance over years spanning pre- and post-cardiac surgery distinguishes sphingomyelin deposition from postoperative or cardiac-related edema. The presence of granulomas causing air trapping may be coincidental in the histoplasma-endemic geographic region.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Intimal Sarcoma of the Pulmonary ArteryNeoplastic
- Massive Unilateral Pulmonary Embolism Mimicking Pulmonary Artery SarcomaVascular
- Plexiform Neurofibroma of the MediastinumNeoplastic
- Milk of Calcium Pleural CollectionsOther
- Niemann-Pick Disease Type B — Septal Thickening and Crazy PavingMetabolic
- Achalasia with Aspiration Lipoid Pneumonia and Crazy PavingOther
- Angio-Invasive Aspergillosis with Air Crescent Sign (Doll's Eyes)Infection
- Lymphoid Interstitial Pneumonia (LIP) with Concurrent MALT Lymphoma in Sjogren's SyndromeNeoplastic
See all cases from September 8, 2017 →