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Start free with EleplanCongenital pulmonary airway malformation type 2
ORPHA:280840Clinical subtype
Also called CCAM type 2 · CPAM type 2 · Congenital cystic adenomatoid malformation of the lung type 2 · Congenital cystic adenomatous malformation of the lung type 2 · Congenital cystic disease of the lung type 2
What it is
A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue, consisting of small cysts of less than 2 cm in diameter. The lesions have intracystic communications, can be connected to the tracheobronchial tree, and are usually unilateral, involving a single lobe. The condition often presents with respiratory distress in the neonatal period or in infancy. It is frequently associated with other severe congenital anomalies, such as renal agenesis or dysgenesis, pulmonary sequestration, or cardiac abnormalities.
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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