Congenital Cystic Adenomatoid Malformation ICD 10 coding tips

Congenital Cystic Adenomatoid Malformation (CCAM) occurs due to an abnormality in lung developement. CCAM has different types of lesions from types 0-4.  Some of the lesion are generally associated with the overgrowth of the terminal bronchioles. It is now also called as Congenital pulmonary airway malformation (CPAM). An abnormal growth of cystic lung tissue replaces the entire lobe of lung, which causes CPAM. This leads to malfunction of the normal lung tissue. The cause for CPAM is still unknown. 
Congenital Cystic Adenomatoid Malformation ICD 10 coding tips



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Congenital Cystic Adenomatoid Malformation Lung

The cystic lesions of lung occur rarely. It is the most common congenital malformation of the lower respiratory tract, hence called as CCAM and bronchopulmonary sequestration (BPS). It occurs mostly in 1 in every 30,000 pregnancies. There are four types of Congenital Cystic Adenomatoid Malformation lung. Let us check all these types of CCAM.

Type 0

This type of CCAM occurs in the trachea or bronchus. This type of cyst are small and rare.

Type 1

This is the most common type of CCAM. These type of CCAM has small number of large cyst. This type of CCAM arises from the distal bronchus or proximal bronchiole.


Type 2

 These type of CCAM arises from the terminal bronchioles. These are small cysts which are not easy to distinguish from the surrounding tissue. These type of CCAM occur in 15% to 30% cases. These are lined by ciliated cuboidal or columnar epithelium, and elements of bronchioles or alveoli may be seen

Type 3

These type of CCAM arises from acinar-like tissue.  These type of cysts are also too small and occurs in 5% to 10% of the cases. These tissues are acinar in nature.

Type 4

These type of cysts are large enough of approximately 10 cm. These type of CCAM can be associated with malignancy.


 ICD 10 Congenital Cystic Adenomatoid Malformation 

We have a ICD 10 code for Congenital cystic lung, Q33.0, which is used for CCAM as well. There are many synonyms which can used for ICD 10 code Q33.0. Below are the common diagnosis which are use for coding Q33.0.

  • Bronchogenic cyst
  • Congenital bronchogenic cyst
  • Congenital cystic adenomatoid malformation of lung
  • Cystic adenomatoid malformation
  • Cystic lung, congenital
  • Single lung cyst

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