• Journal of Chest Surgery
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• v.12 no.2
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• pp.89-92
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• 1979

Congenital multiple cystic disease of the lung is a loosely knitted clinical group of disease and shows various clinical and laboratory findings. It is hard to find out definite differences between the bronchogenic cyst and congenital multiple cystic disease of the lung in the embryologic developing process but we can accept the idea, the embryologic developing process is similar one. An 18 years old female patient had left lower lobe bronchiectasis and Rt. Mid. and lower lobe congenital multiple cystic disease of the lung. In BNUH chest surgery department, we managed this patient successfully by doing staged bilateral lung lobar resection.

• Journal of Chest Surgery
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• v.24 no.8
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• pp.819-823
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• 1991

The congenital cystic adenomatoid malformation of the lung is a rare disease, and is one of the most common congenital lung diseases which require prompt surgical intervention. The prognosis depends on its tissue type, prompt diagnosis and surgical intervention. The lesion consists of enlarged, variable sized multiple cyst with overgrowth of terminal bronchioles, like hamartoma. This disease can be associated with other vascular anomalies or other congenital defect especially in type II lesion We recently experienced one case of congenital cystic adenomatoid malformation The patient was 2 months old infant who showed respiratory distress without associated anomaly. After right upper lobe lobectomy, the patient was recovered uneventfully.

• Journal of Chest Surgery
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• v.10 no.1
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• pp.118-123
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• 1977

Congenital cystic disease of the lung is very rare and controversial disease. But in general is regarded as developmental anomaly. Occasionally failure of the primitive lung bud to develop combined with cardiac anomaly had been reported but it was very rare. Recently a case of cystic lung disease combined with pulmonic valvular stenosis was seen in this clinic with clinical pictures of nonspecific respiratory infection and X-ray finding very similar to that of far advanced pulmonary tuberculosis, destroyed left lung. This case was treated by closed pulmonic valvulotomy and left side pneumonectomy successfully.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.805-811
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• 1996

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.

• Journal of Chest Surgery
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• v.49 no.3
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• pp.210-213
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• 2016

Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.

• Journal of Chest Surgery
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• v.27 no.11
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• pp.930-937
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• 1994

Pulmonary sequestration, congenital cystic adenomatoid malformation[CCAM], infantile lobar emphysema[ILE], and bronchogenic cysts are four congenital lesions that show abnormal cystic areas within the lung field in early life. They share similar embryologic and clinical characteristics, Therefore they are sometimes difficult to make differential diagnosis each other, and all require surgical treatment. From 1984 to 1993, 20 patients underwent surgical corrections under these diagnostic categories[10 bronchogenic cyst, 4 pulmonary sequestration, 4 CCAM, and 2 ILE] in the department of thoracic & cardiovascular surgery, Inje University, Pusan Paik Hospital. There were 9 females and 11 males, Ages ranged from 26 days after birth to 69 years. Among them 5 cases of bronchogenic cyst were found out incidentally, but remained all 15 cases were noted as symptomatic cases. Recurrent pulmonary infections, respiratory distress and cough with cystic lesions in chest film were the main characteristics of them. Computed tomography and aortography were available for diagnostic conformation. For all the cases surgical resection were performed: 1 pneumonectomy, 2 bilobectomy, 9 lobectomy, 7 cyst resection and 1 mass[extralobar pulmonary sequestration] resection. All surgical treatments were well tolerated with no physical limitation. There was no operative mortality, and only one postoperative complication[empyema thoracis]. All patients were followed up ranging from 4 months to 9 years. A clinical awareness of these related lesions is important for prompt diagnosis and effective surgical treatment.

• Journal of Chest Surgery
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• v.36 no.4
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• pp.285-288
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• 2003

Cytomegalovirus (CMV) pneumonitis leading to inflammation and obstruction of the tracheobronchial tree may cause the cystic changes in the lung. We performed segmentectomy of lung under the diagnosis of congenital cystic lung disease in an infant of 2 weeks presenting severe respiratory failure. Histology and serology confirmed congenital CMV bronchiolopneumonitis.

• Journal of Chest Surgery
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• v.26 no.8
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• pp.650-653
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• 1993

Congenital cystic adenomatoid malformation [ C.C.A.M.] is rare, but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced an infant with C.C.A.M. Type II. The patient was a 7-month-old male, and had been suffering from coughing and fever since 3 months ago. The symptom were not improved with medical treatment and then, exploratory thoracotomy was performed under the impression of infected C.C.A.M. or other congenital cystic disease.The left lower lobectomy was performed, and the postoperative course was uneventful.

• Journal of Chest Surgery
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• v.23 no.4
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• pp.698-706
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• 1990

Congenital Cystic Lung Disease is a spectrum of closed related anomalies that arise during an early stage of embryonic lung bud maturation-namely bronchogenic cyst, congenital lobar emphysema, pulmonary sequestration and congenital cystic adenomatoid malformation. And they show similar surgical strategies. So they are called as the term bronchopulmonary-foregut malformations, firstly proposed by Gerle[1968]. From Aug. 1979 to Aug 1989, 47 patients were operated upon on Dept. of Thoracic & Cardiovascular Surgery at the CUMC. There were 21 females and 26 males ranging in age from age of 21 day to age of 56 year [15 cases under 15 years old]. 30 patients had bronchogenic cysts - 23 of intrapulmonary type, 7 of mediastinal type in location. Affected lobes and locations were as follows: 11 in upper lobe, 3 in middle lobe, 11 in lower lobe and anterosuperior, middle, and posterior mediastinal type were 3, 2, 2 respectively. There were 9 pulmonary sequestrations[all intralobar type] with the distribution of 5 in right lower lobe and 4 in left lower lobe. And associated anomalies were presented with arterial supply originating from thoracic aorta[8 cases], abdominal aorta[1 case] and with venous drainage into azygos vein[1 case]. They all were operated upon lower lobectomy [8 case], pneumonectomy[1 case] in case of pulmonary hypoplasia Congenital lobar emphysema and congenital cystic adenomatoid malformation had 4 cases respectively. Their affected lobes were as follows: the former were 3 in upper lobes, 1 in middle lobe and the latter were 3 in upper lobe, 1 in lower lobe. They were treated with lobectomy and segmentectomy. Diagnosis was aided by chest X - ray, bronchography, aortography, DSA and CT scan, They all were confirmed by pathologic exams. There were no hospital death but few minor morbidities such as, atelectasis-pneumonia[2], wound infection[2], prolonged chest tube placement[2]. We experienced surgical treatments of 47 cases for 10 years and reported them with literature review.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.711-717
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• 2010

Purpose: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. Methods: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. Results: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. Conclusion: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.