Congenital pulmonary airway malformation type 3

Orpha code: 280847OMIM code:

Definition

A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue consisting of numerous microcysts of less than 0.5 cm in diameter. The lesions have intracystic communications, can be connected to the tracheobronchial tree, and are usually unilateral, involving an entire lobe. The condition may be associated with polyhydramnios, fetal hydrops, and stillbirth, or present with severe respiratory distress in the neonatal period.

Disease data
Classification

Clinical subtype

Synonyms
CCAM type 3
CCAM typu 3
CPAM typu 3
Wrodzona choroba torbielowata płuc typu 3
Wrodzona torbielowatość gruczolakowata płuc typu 3
CPAM type 3
Congenital cystic adenomatoid malformation of the lung type 3
Congenital cystic adenomatous malformation of the lung type 3
Congenital cystic disease of the lung type 3
ORPHA code
280847
OMIM code
-
ICD10 code
Q33.0
ICD11 code
-

No additional description.

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