Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Congenital pulmonary airway malformations (CPAM), type II.
- Radiologic Findings
- Chest radiograph shows a mass-like increased opacity in the right upper lobe. Chest CT image shows a patchy area of consolidation accompanying low attenuation within it in the right upper lobe. This lesion showed as the multiple cystic lesions on the chest CT scanned on 8 years ago.
- Brief Review
- A congenital pulmonary airway malformation (CPAM), historically referred to congenital cystic adenomatoid malformations (CCAM), is a rare developmental, non-hereditary, hamartomatous abnormality of the lung. CPAM constitutes up to 25% of congenital cystic lung lesions. 80~85% of cases are diagnosed in the first 2 years of life, rarely, the presentation is delayed until adulthood. Clinically, adults most frequently present with recurrent or persistent pulmonary infections. Other modes of presentation include hemoptysis, hemothorax, pneumothorax, pyopneumothorax, shortness of breath or as an incidental finding on a chest radiograph.
Radiological abnormalities range from a soft-tissue mass containing single or multiple air-filled cysts of varying sizes to a solid homogeneous mass that may be mistaken for a lung abscess or pneumonia.
After the morphologic description in 1975, Stocker et al. classified CPAM into three subtypes based on the clinical, gross and histological features. Type I is the most commonly encountered (50~70% of cases) with single or multiple large cysts (2 cm in diameter). Type II lesions (approximately 40% of cases) are characterized by multiple small cysts less than 2 cm in diameter (rarely larger). Type III lesions are rare (approximately 3% of cases). It usually shows as a bulky, solid mass.
Because the majority of cases are associated with recurrent infection and risk for development of carcinoma, Treatment of CPAM is usually surgical, involving complete resection of the relevant part of the lung.
- References
- 1. Huang HJ, Talbot AR, Liu KC,et al. Infected cystic adenomatoid malformation in an adult. Ann Thorac Surg. 2004;78(1):337-9.
2. Herrero Y, Pinilla I, Torres I, et al. Cystic adenomatoid malformation of the lung presenting in adulthood. Ann Thorac Surg. 2005;79(1):326-9.
3. Oh BJ, Lee JS, Kim JS, et al. Congenital cystic adenomatoid malformation of the lung in adults: clinical and CT evaluation of seven patients. Respirology. 2006;11(4):496-501.
4. Khan NU, Jones MT, Greaves M. Case report: Congenital cystic adenomatoid malformation of an entire lung in a 33-year-old man: a case report and review of the literature. Br J Radiol. 2008;81(971):e276-8.
- Keywords
- Airway, Congenital, Bronchial abnormlity,