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Start free with EleplanMounier-Kühn syndrome
ORPHA:3347Clinical syndrome
Also called Congenital tracheobronchomegaly · Idiopathic tracheobronchomegaly · Tracheobronchomegaly
What it is
A rare congenital respiratory disorder characterized by marked dilatation of the trachea and proximal bronchi that leads to impaired airway secretion clearance and recurrent lower respiratory tract infections.
Key facts
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Clinical syndrome
Signs and symptoms
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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