Pediatr Allergy Respir Dis.  2001 Mar;11(1):51-60.

Congenital Cystic Adenomatoid Malformation of the Lung: Analysis of 17 Cases

• Jung JA
• Jeon KH
• Park YM
• Lee JS
• Chung EH
• Nam SY
• Jang KY
• Lee SI
• Kim BK
• Han JH
• Kim JG
• Ahn KM

Abstract

PURPOSE
Congenital cystic adenomatoid malformation of the lung(CCAM) is characterized by an excessive overgrowth of terminal bronchiolar-type structures with a lack of mature alveoli. This study was performed to evaluate the manifestation among different pathologic classification.
METHODS
Seventeen patients admitted to the Samsung Medical Center from January 1995 to June 2000, were investigated for their clinical characteristics, radiologic findings, timing of treatment and post-operative outcome in each type.
RESULTS
1) Seven patients have type 1, while 9 cases were type 2. One case was diagnosed as having mixed type. Mean ages at diagnosis are 7 years 4 months. 2) 4 cases had experienced respiratory diseases that are pneumonia(3 cases), empyema(2 cases) and lung abscess(2 cases). 3) The most common clinical manifestations are fever and cough, followed by chest pain, dyspnea, cyanosis, weak crying and moaning. 4) The left lung(10 cases), especially left lower lobe(8 cases) is involved more frequently than right lung. 5) Associated anomalies are pulmonary sequestration, pectus excavatum, diaphragmatic hernia. 6) All lesions were surgically removed and all of patients survived. In our cases, there was no difference in outcome by age at operation.
CONCLUSION
In this study, CCAM show various clinical manifestations, and the outcome is good irrespective of their pathologic type and age at operation. Probably this is due to few associated anomalies in our cases, but it is not clear whether this is common finding in Korea. Our findings suggest that CCAM is treated successfully by surgical removal which can be delayed unless respiratory symptoms are evident.

Keyword

Congenital cystic adenomatoid malformation

MeSH Terms

Bronchopulmonary Sequestration
Chest Pain
Classification
Cough
Crying
Cyanosis
Cystic Adenomatoid Malformation of Lung, Congenital*
Diagnosis
Dyspnea
Fever
Funnel Chest
Hernia, Diaphragmatic
Humans
Korea
Lung*