• Journal of Chest Surgery
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• v.14 no.4
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• pp.350-353
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• 1981

The pulmonary sequestration is an uncommon congenital anomaly characterized by the presence of a part of lung tissue which is supplied by an aberrant artery from the aorta or its branch and usually has no communication with the normal bronchial tree. It was first presented by Hubber in 1777 and presented in details by Pryce in 1946. We present a case of extralobar pulmonary sequestration experienced recently with a case of intralobar type experienced in 1962. The patient was 11 year old male with the complaint of chronic productive cough. Serial chest films showed a large cyst with or without the air-fluid level on the posterobasal segment area of the left lower lobe. Bronchography showed no definite communication between the cyst and bronchial tree. On operation, the cystic lesion was supplied by an aberrant artery from the descending thoracic aorta 5 cm above the aortic hiatus and was sited at the posterobasal segment area of the left lower lobe. We performed the sequestrectomy and the sequestration was surrounded by its own pleura, 6.8x3.9x3.2 cm in size, contained the pale brown mucoid secretion in a large cyst and showed the primitive alveolar structure of the wall. The aberrant artery was 1 -5 cm long, 0.3 mm in internal diameter and arterio-sclerotic. We also compared 6 cases of collection, 5 intralobar and 1 extralobar type, presented in Korea.

• Journal of Chest Surgery
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• v.17 no.1
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• pp.118-124
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• 1984

Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

• Korean Journal of Animal Reproduction
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• v.23 no.1
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• pp.63-68
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• 1999

A 4-year old Korean-native cow of third parturition delivered an deformed calf with congenital anasarca and hypoplasia of the extremity at Muan County in Chonnam Province. At parturition, the dam showed amniotic fluid approximately two times more than that at the normal and also occurred severe dystocia. The anomaly died before parturition was 82 cm long and weighed 25 kg. It was difficult to recognize the neck and face of the anomaly due to severe edema and the stretched tongue through mouth was found. The shape of the extremity was recognizable and the systemic hypoplasia of the body with marked edema was found. At necropsy, there was a large amount of serous fluid both in pleural and peritoneal cavity. Also the fluid was contained some of yellowish granular materials and diffusely was fulfilled in all subcutaneous tissue. There was a partial opening in diaphragm which was resulted in the protrusion of approximately half of liver toward peritoneal cavity. There were two kidneys (15${\times}$21 and 13${\times}$18 cm) on the left which were enlarged and relatively larger than the right one (13${\times}$9 cm). In addition, there were found slight to moderate hepatomegaly and splenomegaly with slight congestion, and diffuse edema of intestinal serosa. Lung (32${\times}$49 mm) with marked hypoplasia was smaller than the size of heart (56${\times}$45mm). Serological test to the dam showed relatively high antibody titer (＞64) to Akabane virus which might be involved in the outbreak of the deformed Korean-native calf as one of many causative agents.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.335-342
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• 2008

Background: Congenital cystic diseases of the lung are uncommon, and they share similar embryogenic and clinical characteristics. But they are sometimes vary widely in their presentation and severity. Therefore they are often difficult to make different diagnosis each other, and all require surgical treatment. Material and Method: From 1993 to 2006, 38 patients underwent surgical procedures under these diagnostic categories in the Depart. of Thoracic and. Cardiovascular Surgery, Busan-Paik Hospital, College of Medicine, Inje University. And we retrospectively reviewed these patients' charts for clinical presentations, surgical procedures, pathologic findings and postoperative morbidity and mortality. Result: There were 22 males and 16 females, ages ranged from 1 month after birth to 51 years and mean age was 20.8 years. The main symptoms were 19 fever, cough, sputum production due to recurrent infection, 7 dyspnea, 8 chest discomfort, 4 hemoptysis, but eight patients were asymptomatic. Computed tomography was chosen as diagnostic modalities and available for operation plan for all of patients. For all the cases, surgical resection were performed. Lobectomy was performed in 28 patients, simple excision (resection) in 8 patients, segmentectomy or wedge resection in 2 patients. There were 10 pulmonary sequestrations, 15 congenital cystic adenomatoid malformations (CCAM), 11 bronchogenic cysts, and 2 congenital lobar emphysemas. They all were confirmed by pathologic exams. The complications were 6 wound disruption or infection, 2 chylothorax, 1 ulnar neuropathy, but all of them were resolved uneventful. There was no persistent air leakage, respiratory failure, operative mortality and recurrence. Conclusion: We performed immediate surgical removal of congenital cystic lung lesions after diagnosis and obtained good results, so reported them with literature review.

• Journal of Chest Surgery
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• v.40 no.11
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• pp.802-804
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• 2007

Although intrathoracic liver in association with a congenital diaphragmatic hernia has been well documented, the finding of intrathoracic ectopic liver tissue in the presence of an intact diaphragm is an extremely rare congenital anomaly. We have experienced a case of intrathoracic ectopic liver without any diaphragmatic hernia. A 37-year-old woman was admitted for the treatment of an incidentally detected right lung mass, A chest computed tomography scan revealed a right lower lobe lung mass close to the diaphragm, and this was suspicious for bronchial carcinoid tumor. Upon surgery, 2 round solid masses $3.5{\times}3.5 cm$ and a $2.0{\times}2.0 cm$ in size were noted, with their bottoms attached to the diaphragm dome. The masses were completely resected. Histologically, they were confirmed to be intrathoracic ectopic livers. The patient had an uncomplicated postoperative course.

• Journal of Yeungnam Medical Science
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• v.20 no.1
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• pp.71-78
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• 2003

Unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly that is frequently associated with other cardiovascular anomalies first reported by Fraentzel in 1968. Most patients who have no associated cardiac anomalies have only minor or absent symptoms. We experienced a case of isolated UAPA in a young female presenting hemoptysis. The chest radiograph showed a small left lung volume and high resolutional CT of chest showed multiple subpleural nodules and centrilobular nodules in parenchyma. The video-assisted thoracoscopic biopsy revealed diffuse dilated vessels in visceral pleura. The pulmonary angiogram confirmed the absence of the left main pulmonary artery.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.364-367
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• 2009

Retroperitoneal pulmonary sequestration is an extremely rare congenital malformation. It is more frequently diagnosed in the antenatal period due to routine ultrasonic examinations that are conducted for a fetus or during the first 6 months of life, although retroperitoneal pulmonary sequestration is incidentally discovered in adults on rare occasions. Because the location and radiological findings of retroperitoneal pulmonary sequestration are very similar to those of another retroperitoneal masses, retroperitoneal pulmonary sequestration, although they are very rare, should be included in the differential diagnosis of a retroperitoneal suprarenal mass. Although fine needle aspiration may be considered as an aid for making the preoperative diagnosis, surgery remains the treatment of choice for symptomatic lesions and this surgery is associated with excellent results and a good prognosis.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.171-175
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• 2006

Minimally invasive thoracic surgery has been one of the most important surgical advances recently. Congenital cystic adenomatoid malformation of the lung is a relatively rare anomaly and is clearly associated with various congenital anomalies such as pectus excavatum, cardiac and pulmonary vascular lesions. We have experienced a case that was treated with minimal invasive methods for congenital cystic adenomatoid malformation involving in the right lower lobe and pectus excavatum in a 5-year-old boy. We simultaneously performed thoracoscopic right lower lobectomy and Nuss procedure of pectus excavatum using a substernal steel bar. Therefore, a minimally invasive surgical treatment for this diseases is feasible and cosmetically excellent.

• Tuberculosis and Respiratory Diseases
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• v.42 no.5
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• pp.767-771
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• 1995

The bronchial artery-pulmonary vein malformation should be called the systemic artery-to-pulmonary vein arterioveonus malformation in the lung. Although pulmonary arteriovenous malformation has been well documented in intrapulmonary arteriovenous malformation, the systemic artery-to-pulmonary vein arteriovenous malformation is rare. Most patients with systemic artery-to-pulmonary vein arteriovenous malformation is asymptomatic and the diagnosis of these anomaly may be done by continuous murmur or abnormal chest X-ray on the physical examination. The pathogenesis of this condition is congenital malformation which explains these anastomoses between the pulmonary vein and accessory brachial arteries and acquired malformation which explains development of new blood vessel to supply large enough to cause significant systemic-pulmonary shunts due to inflammation secondary to infection, trauma, or previous surgery. We experienced a case of the bronchial artery-pulmonary vein malformation which was detected on angiography in 20-year-old women whose chief complain is hemoptysis. This massive hemoptysis was controlled by selective brachial artery embolization with Gelfoam and Ivalon particles.

• Tuberculosis and Respiratory Diseases
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• v.70 no.1
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• pp.79-83
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• 2011

Pulmonary sequestration is a rare anomaly, in which a local area of a lung is supplied separately by an anomalous artery that arises from the aorta or one of its branches. Infection, mainly bacterial, is a major complication of sequestration. We report the case of a 17-year-old male patient, who presented with cough and fever. The contrast-enhanced chest computer tomomgraphy (CT) scans revealed an aberrant artery that originated from the descending thoracic aorta. He underwent a left-lower lobectomy. Macroscopically, the abnormal segment presented as multiple heterogenous cystic and solid lesions, and the cysts were filled with mucoid and pus-like material. Histology showed that the pulmonary parenchyma had been replaced by caseating epitheloid granulomas. The mycobacterial culture of his sputum was positive. On the basis of these results, the diagnosis of tuberculosis was established. The patient was treated with anti-tuberculous medication for 6 months, and 1 year later, his clinical status remained excellent.