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Answer to Case of the Week: Jun 10-Jun 17, 2010

2 diffferent patient with same diagnosis?


 




One year old shows a large cyst and one month old shows multiple cysts.

Diagnosis: Congenital Pulmonary Airway Malformation ( CPAM)

Old terminology is Congenital Cystic Adenomatoid Malformation ( CCAM)

 

CPAM is developmental hamartomatous abnormality of the lung with adenomatoid proliferation of cysts resembling bronchioles.

 

CPAM communicates with bronchial tree at birth and fill with air early in life.

 

CPAM has no lobar predilection unlike congenital lobar emphysema.

 

Most lesions are confined to single lobe.

 

Incidence 1 per 100,000 births

 

TYPES: 3 depending on the size of the cysts.

 

Type I: 50% 1 or more large (2-10cm ) cysts.

 

Type 2: 40%  Numerous small cysts of uniform size from 0,5 -2 cm. Associated with other congenital anomalies ( renal, skeletal, intestine and cardiac)

 

Type 3: 10% Appears solid on gross imaging and inspection but have microcysts.

 TAKE HOME MESSAGE:

Small risk for malignant degeneration –Rhabdomyosarcoma and bronchoalvelolar cell carcinoma.

    

Residents Submitting Correct Diagnosis - Case of the Week
Radiology
Pediatrics
VCU Resident
  • Judson Frye
  • Jonathan Ha
  • Kenny Uy
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    Disclaimer: This information is intended solely for resident review of presented cases which may or may not be pathologically proven. Information is derived from a number of published sources of varying reliability and does not represent original research from the institution. It is not intended to be comprehensive and should therefore not substitute for careful review of the literature.