Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung

14세 여아에서 발견된 선천성 낭종성 선종성 기형 1예

  • Lee, Myung In (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Sohn, So Hee (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Lee, Dae Joon (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Ha, Dong Yul (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Jee, Young Koo (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Lee, Kye Young (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Kim, Keun Youl (Department of Internal Medicine, Dankook University, College of Medicine) ;
  • Choi, Young Hi (Department of Radiology, Dankook University, College of Medicine) ;
  • Cho, Jeong Hi (Department of Pathology, Dankook University, College of Medicine) ;
  • Seo, Pil Weon (Department of Thoracic surgery, Dankook University, College of Medicine) ;
  • Kim, Sam Hyun (Department of Thoracic surgery, Dankook University, College of Medicine)
  • 이명인 (단국대학교 의과대학 내과학교실) ;
  • 손소희 (단국대학교 의과대학 내과학교실) ;
  • 이대준 (단국대학교 의과대학 내과학교실) ;
  • 하동열 (단국대학교 의과대학 내과학교실) ;
  • 지영구 (단국대학교 의과대학 내과학교실) ;
  • 이계영 (단국대학교 의과대학 내과학교실) ;
  • 김건열 (단국대학교 의과대학 내과학교실) ;
  • 최영희 (단국대학교 의과대학 방사선과학 교실) ;
  • 조정희 (단국대학교 의과대학 병리학 교실) ;
  • 서필원 (단국대학교 의과대학 흉부외과학 교실) ;
  • 김삼현 (단국대학교 의과대학 흉부외과학 교실)
  • Published : 1996.10.30
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Abstract

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

저자들은 14세 여아에서 호흡곤란과 흉통이 발생하였으나, 긴장성 기흉등으로 잘못 인식되었던 선천성 낭종성 선종성 기형(CCAM)을 진단하여 수술적 방법으로 치료한 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

Acknowledgement

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