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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 - Tuberc Respir Dis (Seoul) (2012)

Bottom Line: CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case.A 54-year-old female presented with chronic cough and blood-tinged sputum.Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea.

ABSTRACT

Unlabelled: 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.

No MeSH data available.


Related in: MedlinePlus

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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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).

Mentions: To diagnose and treat this lesion, the patient underwent a right middle lobectomy and a medial basal segmentectomy of the right lower lobe via a right posterolateral thoracotomy. The cystic malformation was excised en bloc along with the right lower lobe. The operation was completed without complications. On gross inspection, the specimen showed multilobular cystic lesions ranging in size from 0.3 to 1.8 cm in diameter, separated by grossly unremarkable pulmonary parenchyma. The diameter of the whole cystic lesion measured 12.5×11.0×5.2 cm3, and there was yellowish white mucous and necrotic material in the medial basal segment of the right lower lobe. Histologically, the right lower lobe was seen to contain a multilocular cystic lesion lined by stratified ciliated columnar epithelium, with underlying cartilage and mucus-secreting glands. There was marked infiltration of neutrophils, lymphocytes, and plasma cells in the cyst wall, with focal calcification and a proliferation of small blood vessels. These findings were consistent with type I CCAM (Figure 4A). The bronchus of the right middle lobe of the lung was cystically dilated and lined by pseudostratified ciliated columnar epithelium. The ciliated bronchus contained mucus material, and the bronchial wall contained underlying cartilage and mucous glands with few calcifications. The alveolar spaces distal to the ectatic bronchus were dilated and there was septal smooth muscle proliferation (Figure 4B). These findings favor a bronchocele. All 24 lymph nodes were unremarkable. Since removal of the anomalies the patient has been followed up in the outpatient-clinic for 2 years without any complications.


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 - Tuberc Respir Dis (Seoul) (2012)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3475458&req=5

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).
Mentions: To diagnose and treat this lesion, the patient underwent a right middle lobectomy and a medial basal segmentectomy of the right lower lobe via a right posterolateral thoracotomy. The cystic malformation was excised en bloc along with the right lower lobe. The operation was completed without complications. On gross inspection, the specimen showed multilobular cystic lesions ranging in size from 0.3 to 1.8 cm in diameter, separated by grossly unremarkable pulmonary parenchyma. The diameter of the whole cystic lesion measured 12.5×11.0×5.2 cm3, and there was yellowish white mucous and necrotic material in the medial basal segment of the right lower lobe. Histologically, the right lower lobe was seen to contain a multilocular cystic lesion lined by stratified ciliated columnar epithelium, with underlying cartilage and mucus-secreting glands. There was marked infiltration of neutrophils, lymphocytes, and plasma cells in the cyst wall, with focal calcification and a proliferation of small blood vessels. These findings were consistent with type I CCAM (Figure 4A). The bronchus of the right middle lobe of the lung was cystically dilated and lined by pseudostratified ciliated columnar epithelium. The ciliated bronchus contained mucus material, and the bronchial wall contained underlying cartilage and mucous glands with few calcifications. The alveolar spaces distal to the ectatic bronchus were dilated and there was septal smooth muscle proliferation (Figure 4B). These findings favor a bronchocele. All 24 lymph nodes were unremarkable. Since removal of the anomalies the patient has been followed up in the outpatient-clinic for 2 years without any complications.

Bottom Line: CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case.A 54-year-old female presented with chronic cough and blood-tinged sputum.Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea.

ABSTRACT

Unlabelled: 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.

No MeSH data available.


Related in: MedlinePlus