• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.116-121
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• 1999

Unilateral hypoplasia of the pulmonary artery is an uncommon anomaly, which commonly develops in combination with congenital cardiovascular defects such as tetralogy of Fallot, patent ductus arteriosus and septal defect of atrium or ventricle, but may also present as an isolated lesion. We have recently experienced a case of the left pulmonary artery hypoplasia in adult by chance of during the general health screen, which diagnosed by chest X-ray, chest spiral CT, lung perfusion and ventilation scan, digital substraction angiogram and bronchoscopy, then presented hereby with the review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.45 no.4
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• pp.896-901
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• 1998

Pulmonary arteriovenous malformation(PAVM) is an uncommon congenital anomaly. As pulmonary arteriovenous malformation is a direct communication between the branches of pulmonary artery and vein which originated from the malformation of capillary development, major disturbances in gas exchange can result. This malformation results in the several symptoms such as dyspnea, hemopyssis, cyanosis, and severe neurologic complaints. However, the most of patients are usually asymptomatic. Selective pulmonary angiography is well known the helpful diagnostic method. Recently, therapeutic embolization has been advocated as the treatment of choice for pulmonary arteriovenous malformations. We report a case of multiple pulmonary arteriovenous malformation, which was detected on the simple chest X-ray and successfully treated with coil embolization in a 19-year-old asymptomatic woman.

• Tuberculosis and Respiratory Diseases
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• v.78 no.4
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• pp.380-384
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• 2015

Systemic arterial supply from the descending thoracic aorta to the basal segment of the left lower lobe without a pulmonary arterial supply is a rare congenital anomaly within the spectrum of sequestration lung disease. The most common pattern of anomalous systemic artery to the lung arises from the descending thoracic aorta and feeds the basal segments of the left lower lobe. We report an extremely rare case of a 29-year-old woman who underwent a successful left upper lobectomy for the treatment of recurrent massive hemoptysis from anomalous bronchial arterial supply to the lingular segment of left upper lobe.

• Tuberculosis and Respiratory Diseases
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• v.68 no.2
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• pp.101-104
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• 2010

Bronchopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract. Most intralobar BPSs are provided with an arterial blood via the thoracic or abdominal aorta but such a supply is rarely found in patients older than 50 years. We report a case of an intralobar BPS with a dual arterial supply from the celiac artery and thoracic aorta in a 50-year-old man presenting with a respiratory tract infection and haemoptysis. To our knowledge, this is the first case report of a BPS supplied by the celiac artery and thoracic aorta in a 50-year-old man.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.718-722
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• 1999

Thymoma is the most common tumor in the compartment of anterior mediastinum. The malignant thymoma is classified into invasive thymoma(category I) and thymic carcinoma(category II). Recently, well-differentiated thymic carcinoma is a proposed category 1.5 used to describe a subset of thymic epithelial tumors, allowing for the existence of intermediate form based on the clinical features and the histological characteristics. Thymic cyst is a congenital or a acquired disorder. Congenital thymic cyst may develop due to failure of the thymopharyngeal duct to obliterate and acquired thymic cyst develops from inflammation(multilocular thymic cyst), or neoplasm(cystic thymoma). Cystic degeneration in thymoma is a relatively frequent but focal event. In rare cases, the process proceeds to the extent that most or all of the lesion becomes cystic. Until now, well-differentiated thymic carcinoma with extensive cystic degeneration has not been reported in our country. We experienced a case of 14 year-old female patient showing extensive cystic degeneration in well-differentiated thymic carcinoma. And so we report it with review of the articles related.

• Tuberculosis and Respiratory Diseases
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• v.50 no.3
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• pp.373-377
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• 2001

An anomalous systemic arterial supply to the normal basal segments of the left lower lobe without sequestration is a rare congenital anomaly. It differs from classical bronchopulmonary sequestration in that the involved lung retains a normal connection to the bronchial tree, although some place this entity exists within the broad framework of pulmonary sequestration. We experienced a case of a woman who presented with a nodular lesion on a chest X-ray. Contrast-enhanced CT diagnosed her as having an anomalous systemic arterial supply to the normal basal segments of the left lower lobe. This case is reported with a brief literature review.

• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.430-434
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• 2008

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.129-135
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• 1999

We describe unusual manifestations of congenital cystic adenomatoid malformation (C.C.A.M.) of the lung, such as movable fungal ball-like intracystic blood clots and hemoptysis, which were detected in previously healthy 23 years-old woman. We identified blood clots only after left upper lobectomy and could not distinguish from fungal ball with radiographic methods. CCAM of the lung, rare and lethal form of congenital pulmonary cystic disease, was initially introduced by Ch, in and Tang in 1949. The histogenesis of this lesion is characterized by polypoid glandular tissue proliferation and overgrowth of mesenchymal elements due to cessation of bronchiolar maturation which occurred in after 16weeks intrauterine period. In 80-95% of reported cases, the lesion was confined to a single lobe and there was no lobe and right and left lung predilection The clinical presentation may be widely variable, ranging from intrauterine fetal death to late discovery in childhood with recurrent pulmonary infection. But there,s no reports which were misdiagnosed with intracystic fungal ball. The treatment choice is lobectomy of affected lobe. There,s a few case reports with rhabdomyosarcoma, bronchiolar cell carcinoma and myxosarcoma arising in CCAM patients. Therefore, early resection is recommended even if asymtomatic cases. We experienced a rare case of CCAM of the lung in 23 years old female, and there were intracystic fungal ball-like movable blood clots in lower portion of left lung. After left upper lobectomy was performed, now she is discharged and followed up without any complications.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1383-1390
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• 1992

This is the result of the annual statistic analysis of thoracic and cardiovascular surgical cases in 1991, Korea. 14,715 cases of surgery[thoracic 8,995/cardiovascular 5,720] were done by 53 institutes replied. The order of frequency of cell type in primary lung cancer was squamous [62.3%] / adeno [23.9%] / small [6.4%] / adenosquamous [3.0%], and in mediastinal tumor, neurogenic[27.l%] / thymoma [27.1%] / teratoma[26.4%] / congenital cystic[12.0%]. Surgery for tuberculosis was decreased to 15.8% of overall infectious disease from the recent 6 year`s average 35.7%. In general thoracic surgery, the single most frequent operation was closed thoracostomy[4,047 cases] for pleural pathology. The ratio of congenital to acquired heart disease was 2:1, and acyanotic to cyanotic was 3:1. The order of frequency of congenital acyanotic heart disease was VSD [45.6%] / ASD [25.6%] / PDA [20.4%] / PS [2.9%], and that of cyanotic heart disease was TOF [42.6%] / PA [12.9%] / TGA [9.9%] / DORV [8.8%]. In 1,364 cases of valvular surgery, single mitral pathology was the most frequent candidate[729 cases, 53.4%]. In 243 cases of coronary surgery, bypassing graft materials were great saphenous vein[41.6%], internal mammary [39.5%], and artificial vessel[18.9%]. There were no specific differences in aortic surgery, assisted device implantation, and antiarrhythmic surgery as compared to previous study. This nation-wide inquiry will be continued and reported annually by KTCS Society.

• Tuberculosis and Respiratory Diseases
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• v.74 no.2
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• pp.70-73
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• 2013

Dyskeratosis congenita is a rare congenital disorder characterized by a triad of reticular pigmentation of the skin, dystrophic nails, and leukoplakia of the mucous membrane. Sometimes it is associated with bone marrow failure, secondary malignancy and interstitial lung disease. Though it is rare, Dyskeratosis congenita is diagnosed relatively easily when clinicians suspect it. It can be diagnosed just by gross inspection with care. Dyskeratosis congenita should be considered as one cause associated with interstitial lung disease. In Korea, interstitial lung disease with dyskeratosis congenita has not been reported. We report a case and review the literature.