Tuberc Respir Dis.  2012 Jun;72(6):501-506.

Congenital Cystic Adenomatoid Malformation with Bronchial Atresia in Elderly Patients

• Kwak HJ
• Moon JY
• Kim SI
• Kim TH
• Sohn JW
• Kim SH
• Shin DH
• Park SS
• Chung WS
• Yoon HJ

Abstract

Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.

Keyword

Cystic Adenomatoid Malformation of Lung, Congenital; Aged; Bronchi; Abnormalities

MeSH Terms

Aged
Bronchi
Cough
Cystic Adenomatoid Malformation of Lung, Congenital
Female
Humans
Infant, Newborn
Lung
Mastectomy, Segmental
Middle Aged
Physical Examination
Sputum
Thoracotomy
Thorax

Figure

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  Figure 1 Simple chest radiography on admission. A patch of opacity is seen in the right lower lung field (arrow).

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  Figure 2 Computed tomography scan of the chest. (A) Multiple large air-filled cysts consistent with a congenital cystic adenomatoid malformation are evident in the right lower lobe (arrow). (B) An oval-shaped opacity of the distal right middle lobe bronchus can be seen in the low-attenuated right middle lobe with no clear connection between the lesion and the tracheobronchial tree (arrow).

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  Figure 3 Bronchoscopic examination. The right middle lobe (RML) bronchus is not seen on bronchoscopic examination and there is a suspicious dimpling lesion (arrow) in the proximity of the RML bronchus.

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  Figure 4 Pathologic examination of the resected lesions. (A) Right lower lobe. Multilocular cystic lesions lined by stratified ciliated columnar epithelium with underlying cartilage and mucus-secreting glands can be seen, together with infiltration of neutrophils, lymphocytes, and plasma cells. These findings are consistent with Stocker's type I congenital cystic adenomatoid malformation and bronchial atresia (H&E stain, ×400). (B) Right middle lobe. The enlarged bronchus and dilated distal alveolar spaces reveal bronchial atresia. A cystically dilated bronchus lined by pseudostratified ciliated columnar epithelium can be seen (H&E stain, ×100).

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