• 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.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.796-799
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• 2006

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

• Journal of Chest Surgery
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• v.33 no.7
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• pp.594-596
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• 2000

Congenital cystic adenomatoid malformation and Extralobar Pulmonary sequestration are very rare congenital anomalies. We experienced a 4 year-old female patient who had Congenital cystic adenomatoid malformation in her lower lobe of left lung. We accidently found extralobar pulmonary sequestration associated with Congenital cystic adenomatoid malformation at operation field. The resection of the left lower lobe and the extralobar pulmonary sequestration were performed. The arterial supply of the extralobar pulmonary sequestration was one anomalous artery arised from the thoracic aorta. The Venous drainage of expralobar pulmonary sequestration was intercostal vein into the azygous vein. The patient was discharged without any problem.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.1018-1023
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• 2007

Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary and cerebral AVMs and a familial occurrence.

• Neonatal Medicine
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• v.18 no.2
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• pp.404-408
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• 2011

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

• Journal of Chest Surgery
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• v.30 no.5
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• pp.493-500
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• 1997

We performed this study to evaluate hepatic venous drainage in atrial isomerism by MR and the clinical significance of anomalous hepatic venous return in total cavopulmonary shunt operation. Numbers and locations of hepatic veins in twenty-two patients with isomerism(thirteen with right isomerism and nine with left isom rism) were evaluated by MR. Operative procedure of hepatic veins and postoperative arterial oxygen saturation were compared with hepatic vein connection in six patients after total cavopulmonary shunt operation. Among nine patients with left isomerism, hepatic venous return was totally anomalous via a single opening in eight, and via two separate openings in one. Among thirteen patients with right isomerism, partial anomalous hepatic venous connection directly to the atrium was seen in four. One showed total anomalous hepatic venous connection to atrium through one opening. Total cavopulmonary shunt operation was performed in 6 patients. Hepatic veins were connected to pulmonary arteries in four patients who had one atrial opening of hepatic vein andlor IVC, or two ipsilateral atrial opening of hepatic veins and IVC. In conclusion, hepatic vein drainage to atrium is variable in atrial isomerism. MR is useful for evaluation of hepatic vein drainage in atrial isomerism and surgical pla ning.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.355-360
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• 2009

Background: Benign teratoma is mostly asymptomatic, but this tumor rarely ruptures into the adjacent structure such as the pleural space, pericardium, lung parenchyma or tracheobronchial tree. Thus, it is important to differentiate ruptured teratoma from unruptured teratoma. This study evaluated the difference between ruptured and unruptured benign teratoma. Material and Method: Twenty-four cases of surgically resected benign teratomas were reviewed retrospectively. The clinical symptoms, chest CT findings and operative findings of the ruptured teratoma were compared with those of the unruptured teratoma. Especially, the tumor size, wall thickness, location of the mass, internal septation, homogeneity, calcification and ancillary findings were evaluated on CT. Result: Of the 24 patients, 7 patients were diagnosed with ruptured teratoma. Severe symptoms were more commonly found for ruptured teratoma than for unruptured teratoma. The ruptured teratoma had a tendency to display calcification and such ancillary findings as collapse or consolidation of the lung parenchyma. For the ruptured teratoma, the resection was performed by sternotomy or thoracotomy, and more lung resection was included. Conclusion: Calcification within the mass and changes in the lung parenchyma on the preoperative CT findings can be diagnostic signs of a ruptured teratoma. The demonstration of ruptured teratoma is important not only for making the early diagnosis, but also for the surgical planning.

• 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.34 no.3
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• pp.235-239
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• 2001

배경: 폐격리증, 선천성 낭성 유선종 기형, 기관지 낭종 선천성 엽기종 및 낭성 기관지 확장증 등의 폐에 발생하는 선청성 낭성 질환은 않으며 유사한 발생학적 및 임상적 양사을 가진다. 대상 및 방법: 1972년부터 1999년까지 본원 흉부외과에서 선청성 낭성 폐질환으로 수술받은 46명의 환자를 대상으로 병상 기록을 통한 임상상, 치료 및 병리소견을 검토하였다. 결과: 환자의 평균 연령은 16.2세 였고 남녀비는 17:29였다. 주 증상은 감염에 의한 발령이 11례, 호흡곤란이 10례 그리고 흉통과 가래 등이였고, 증상이 없었던 경우가 13례 였다. 질환은 폐격리증이 13례, 선청성 낭성 유선종 기형이 12례, 기관지성낭종이 12례, 낭성 기관지 확장증이 5례 그리고 선천성 엽기종이 4례였다. 수술은 단순 절계 8례, 폐구역 절제술 6례, 폐엽 절제술 31례 그리고 전폐 전제술 1례를 시행하였고 수술 사망은 폐엽 절제술은 시행한 7세된 기관지 낭종 환자로 수술직후 발생한 뇌경색으로 1례에서 발생하였으며 술후 합병증으로는 폐렴 4례와 지속적 공기누출과 농흉이 각 1례씩 이었다. 결론; 선청성 낭성 폐질환을 가진 환자들은 진단 즉시 수술하여 좋은 결과를 얻었다.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.60-62
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• 2007

Pulmonary hypoplasia is an entity of pulmonary agenesis. Pulmonary agenesis is a rare congenital anomaly, usually diagnosed soon after birth. It is commonly associated with other anomalies, mainly of the cardiovascular systems. Although it may hasten the death of a child, sometimes it is compatible with normal growth. We report a right lateral thoracotomy approach for mitral valve repair in a young woman with pulmonary hypoplasia, in whom preoperative computed tomography showed severe right side shifting of the mediastinum and total collapse of the right lung.