Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Congenital cystic adenomatoid malformation (CCAM)
- Radiologic Findings
- Chest radiography shows multiple variable sized thin walled cysts with air fluid level in left lower lobe. Chest CT scan shows multiloculated thin walled cysts (maximal 35 mm) with air fluid level and surrounding consolidation, mainly confined to LLL posterolateral portion. There is no evidence of abnormal blood supply from a systemic artery.
- Brief Review
- Congenital pulmonary airway malformations (CPAM) historically referred to as congenital cystic adenomatoid malformations (CCAM) is a rare anomaly of the lung. These lesions form masses of maldeveloped lung tissue and are identified most commonly in stillborn infants or newborns with respiratory distress. In adult, CCAM may be an incidental finding or may be accompanied by symptoms related to recurrent respiratory infections. Less common complications include pneumothorax and occasionally, the development of bronchioloalveolar carcinoma, adenocarcinoma, or squamous cell carcinoma. In older children and adults, CCAMs usually appear radiologically as a unilocular or multiloculated cyst or as a complex soft tissue and cystic mass. The CT manifestation in adults typically consist of a unilocular or multiloculated cyst or a complex soft tissue and cystic mass. CCAM have been classified into 5 subtypes (types 0 to 4) by Stocker. This classification was based on the histopathologic findings in the area of the tracheobronchial tree involved by the malformation. Of these, Types I-III have been well described in the imaging literature. Type I CCAM contains one or more cysts larger than 2 cm in diameter, usually surrounded by smaller cysts. In Type II lesions, the cysts are less than 2 cm in diameter. Type III CCAM is usually shows as a bulky, solid mass. This classification system also has prognostic significance. Type I CCAM is associated with the best overall prognosis and may go undetected clinically until later in life. Type II CCAM has an increased incidence of severe cardiac or renal anomalies. Type III CCAM is often a large mass associated with stillbirth or death in the neonatal period. Because the majority of cases are associated with recurrent infection and risk for development of carcinoma, CCAMs are usually removed surgically. Lobectomy is generally required.
- References
- 1. Masashi et al. Congenital pulmonary airway malformation CT-pathologic correlation. J Thorac Imaging 2007;22:149?53
2. Patz EF Jr, Muller NL, Swensen SJ, Dodd LG. Congenital cystic adenomatoid malformation in adults: CT findings. J Comput Assist Tomogr 1995; 19:361-364.
3. William D. Winters and Eric L. Effmann. Congenital masses of the lung: prenatal and postnatal imaging evaluation. J Thorac Imaging 2001;16:196?06
- Keywords
- Airway, Congenital,