• Journal of Chest Surgery
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• v.39 no.3 s.260
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• pp.230-235
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• 2006

Systemic arterialization of lung with/without sequestration (Sequestration/Anomalous Origin of Left Pulmonary Artery, AOLPA) is a rare form of congenital anomalous systemic arterial supply to the lungs. In this anomaly, the arterial supply of one or more arteries of the basal segments of the lower lobe derives from an aberrant vessel arising from the aorta. We report two adult cases of systemic arterialization of normal basal segments of left lower lobe lung with/without sequestration. The one (AOLPA) was treated by left lower basal segmentectomy and the other (Sequestration) by therapeutic angiographic embolization. Based on the favorable follow-up result in our patients, although lobectomy (segmentectomy) is the basic treatment modality, embolotherapy could also be a mode of treatment that could be selectively applied to elderly, infirm patients or high risk patients with poor pulmonary function.

• Journal of Chest Surgery
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• v.51 no.4
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• pp.280-282
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• 2018

Unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly that may present with various symptoms, depending on the nature and severity of other cardiovascular anomalies. Furthermore, contralateral lung surgery in patients with UAPA is extremely rare, and clinical experience is limited. This report describes a case of surgical treatment of contralateral primary lung cancer in a patient with isolated UAPA. A 56-year-old man was diagnosed with primary lung cancer accompanied by isolated UAPA on the contralateral side. He underwent meticulous cardiorespiratory function tests preoperatively. We performed a right lower lobectomy. Although in the immediate postoperative period, the patient suffered from a mild decline in his respiratory function, he recovered uneventfully. The present case shows that preoperative awareness of UAPA and meticulous perioperative management enable contralateral lung surgery to be performed safely.

• Journal of Genetic Medicine
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• v.3 no.1
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• pp.1-4
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• 1999

Scimitar syndrome is a rare congenital anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava. The scimitar vein is usually visible on chest radiographs, but may be obscured by the heart. It is essential for surgical correction to establish the point of drainage of the anomalous vein and associated anomalies. There are recent reports of familial total anomalous pulmonary venous return suggesting heritable forms of this anomaly. Although genetic factors are believed to have important roles in congenital heart disease, few genes involved in heart development have been located. We report a case of familial chromosome 9 inversion with Scimitar syndrome in an offspring who presented with dextrocardia. Evaluation with magnetic resonance cineangiograph imaging demonstrated an anomalous pulmonary vein draining into the inferior vena cava above the diaphragm and hypoplasia of the right lung and the right pulmonary artery. Chromsome analysis showed pericentric inversion of chromosome 9, inv 9 (p13, q21), in the patient and his mother as well. A brief review of the related literature is also included.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.550-552
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• 2010

An accessory cardiac bronchus (ACB) is a rare congenital anomaly of the trachobronchial tree. It rarely presents with symptoms, and is usually diagnosed incidentally by chest CT, bronchoscopy, or during surgery. We experienced a case of accessory cardiac bronchus found incidentally in the bronchus intermedius with lung cancer in the right lower lobe, and surgically removed.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.847-850
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• 1999

Tracheobronchomegaly or Mounier-Kuhn syndrome a is rare disease characterized by marked dilatation of the trachea and main bronchi. It is thought to be due to a congenital anomaly, but is uncertain. It has variable clinical manifestations from causing chronic respiratory infections and bronchiectasis to being asymptomatic for the lifetime. Recently, we experienced a case of Mounier-Kuhn syndrome patient with tracheal diverticulum, who had lung cancer. Our case is reported with literature reviews.

• Journal of Chest Surgery
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• v.40 no.11
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• pp.793-797
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• 2007

A communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital anomaly that is characterized by a fistula between isolated respiratory tissue and the esophagus or stomach, The presence of accessory lung tissue arising from the primitive gastrointestinal tube is a common factor in the development of all forms of bronchopulmonary foregut malformations. Recurrent pneumonia associated with cystic radiographic structures is a characteristic of the condition. Further imaging studies using esophagogram, bronchography, computerized tomography, MRI, and arteriography can help in making a diagnostic evaluation. The treatment is a surgical resection of the involved lung tissue, and fistula closure with a good prognosis. We encountered a case of CBPFM, who presented with an extralobar pulmonary sequestration and bronchogenic cyst communicating with a tubular esophageal duplication that was associated with a complete left pericardial defect.

• 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.43 no.4
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• pp.450-453
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• 2010

CCAM with no other anomalies such as sequestration receives its blood supply from the pulmonary artery. Our case presented with a simple CCAM and no other anomalies but with a feeding artery. Although preoperative evaluation may not show feeding arteries, they may exist in congenital cystic lung diseases.

• Journal of Chest Surgery
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• v.24 no.1
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• pp.83-87
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• 1991

A unilateral pulmonary artery agenesis, which develops occasionally as one of the associated anomalies in congenital cardiovascular defect like as tetralogy of Fallot, is very rare anomaly as an isolated congenital defect. The diagnostic approach for the pulmonary artery agenesis is first suggested by the unique appearance of the involved lung on a routinely checked chest roentgenogram, because most patients are asymptomatic unless pulmonary infection had been superimposed. We have recently experienced a case of left pulmonary artery agenesis, which was diagnosed by perfusion scan, digital subtraction angiogram and then treated by left pneumonectomy in a 9 year-old boy, and presented hereby with the review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.72 no.6
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• pp.507-510
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• 2012

Carbohydrate antigen 19-9 (CA19-9) is a specific tumor marker of the biliary, pancreatic and gastrointestinal tracts. CA19-9 is occasionally elevated in serum in patiens with benign pulmonary diseases such as bronchiectasis, idiopathic interstitial pneumonia or collagen disease-associated pulmonary fibrosis. Intralobar pulmonary sequestration is an uncommon congenital lung anomaly. It is dissociated from the normal tracheobronchial tree and is supplied by an anomalous systemic artery. There have been some reports of elevation of CA19-9 in this lesion. We report a case of intralobar pulmonary sequestration with elevated serum CA19-9 in a 29-year-old man who was diagnosed with bronchiectasia of left lower lung field on general check up. He had no evidence of any malignant disease in pancreatobiliary or gastrointestinal tracts. Elevated serum CA19-9 level might be encountered with benign pulmonary disease such as pulmonary sequestration.