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Start free with EleplanCongenital pulmonary airway malformation type 0
ORPHA:280827Clinical subtype
Also called CPAM type 0 · Congenital cystic adenomatoid malformation of the lung type 0 · Congenital cystic adenomatous malformation of the lung type 0
What it is
A rare subtype of congenital pulmonary airway malformation characterized by global arrest of lung development with small, solid appearing lungs with a diffusely granular surface, histologically featuring bronchus-like structures with smooth muscle, glands, and numerous cartilage plates, embedded in loose, vascular mesenchymal tissue. The condition presents at birth and is incompatible with life.
Key facts
- Age of onset
- Antenatal, Neonatal
- Classified as
- Clinical subtype
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (at birth, Europe)Congenital pulmonary airway malformation
- Inheritance
- Not applicableCongenital pulmonary airway malformation
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
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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