• Journal of Chest Surgery
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• v.37 no.12
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• pp.1015-1018
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• 2004

Lymphangioleiomyomatosis (LAM) is a rare disease in women in childbearing age leading to progressive respiratory failure. LAM is characterized by an abnormal harmartomatous proliferation of smooth muscle cells surrounding the blood vessels, lymphatics and airways in the lung. This proliferation leads to airway obstruction, cystic alveolar change and lymphatic obstruction. Patients present with dyspnea, pneumothorax, cough, chest pain, hemoptysis, and chylous effusion. Although lung transplantation is the only therapeutic modality in end-stage LAM with respiratory failure, there has not been any report of successful treatment in Korea. We report one case of successful left single lung transplantation in a 40 year old woman suffering from end-stage LAM.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.326-329
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• 1999

Pulmonary lymphangioleiomyomatosis is a rare disease occurring almost exclusively in woman of reproductive age. It is characterized by rapid deterioration of respiratory functions and results in death within an average of 10 years. This disease is caused by a progressive proliferation of atypical smooth muscle in the pulmonary lymphatic vessels, blood vessels, and airways leading to the development of distal cystic changes which causes frequent recurrent pneumothoraces. We experienced a case of pulmonary lymphangioleiomyomatosis in a 30-year old woman who had a history of bilateral, recurrent pneumothoraces. The patient underwent lung biopsy through right thoracotomy which revealed the diagnosis of pulmonary lymphangioleiomyomatosis. We report a case with a review of the literature.

• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.323-327
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• 2006

Lymphangioleiomyomatosis (LAM) is a rare disease in women of childbearing ages that eventually leads to respiratory failure. Lung transplantation is the only conclusive therapeutic modality in end-stage LAM. While single-lung transplantation is the preferred operation, the graft failure or recurrence of LAM was reported. We performed a single lung transplantation on a 36-year-old woman suffering from respiratory failure due to lymphangioleiomyomatosis. After a 1-year follow up, the patient was readmitted because of graft failure with collapsed transplanted lung. The lung volume reduction surgery (LVRS), tracheostomy and ventilator care were performed. However, neither the medical nor surgical treatment had any effect. Subsequently, we performed a contralateral single lung re-transplantation and had a good postoperative results.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.360-364
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• 1996

Chylothorax is denned by extravasation of the milky fluid to pleural cavity from the thoracic duct or it's main branches due to operative trauma, congenital lesions, diagnostic procedures, tumor, etc. Another rare cause is diffuse pulmonary Iymphangiomatosis which is uncommon and not well charact rized. We experienced a case of the bilateral chylothorax caused by the diffuse pulmonary Iymphangiomatosis. The patient was at years old girl with symptoms of coughing and febrile sensation, and the chest radiographs showed bilateral pleural effusion and interstitial infiltrates. The laboratory data of the pleural effusion was identified as chile. Uncontrollable with closed tube thoracostomy, division of tHe thoracic duct and biopsy were decided. Biopsy showed anastomosing endothelial lined spaces along the pulmonary Iymphatic routes especially in pleural and interlobar septum, and smooth muscle at the proliferative interstitium of the Iymphatic duct was observed. Postoperatively, chylothorax was controlled with several trial of chemical pleurodesis. Af'leer discharge from the hospital, she was well for ten months follow up.

• Journal of Chest Surgery
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• v.32 no.11
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• pp.1057-1059
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• 1999

Lymphangioleiomyomatosis(LAM) is defined as an abnormal proliferation of smooth muscles in the lung tissue throughout lymphatics, vascular and bronchial structure. A 52-year-old postmenopausal woman was admitted to our hospital for recurrent pneumothorax. She was treated for medroxyprogesterone by LAM 1 month ago. We performed operation of pulmonary partial resection and pleurodesis. The patient is receiving continuous medroxyprogesterone and Leuplin administration, and currently, 6 months after the operation, is still showing good results.

• Tuberculosis and Respiratory Diseases
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• v.47 no.4
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• pp.533-537
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• 1999

Pulmonary lymphangiomatosis is a very rare pulmonary lesion with an aggressive potential that occurs mainly in newborns, infants and young children of both sexes. It is characterized by pulmonary abnormalities of lymphatic system, showing an increased number of complex anastomosing lymphatic channels in the pleura, in the subpleural interlobular septa, and along the bronchovascular lymphatic route and uniformly fatal. We report a case of lymphangiomatosis behaving like lymphangioleiomyomatosis in a 26-year-old woman.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.822-827
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• 2006

Background: Lung transplantation is a definitive therapy for a variety of end stage lung diseases. Since 1996, we have performed thirteen cases of lung transplantation including two retransplantations, and we analyzed the outcomes, complications, and survivals of these patients. Material and Method: We retrospectively analyzed the medical records of thirteen cases from July, 1996 to July, 2005. Result: During the period, 11 patients had undergone 43 lung and heart-lung transplantations, and two patients had retransplantation due to allograft failure. Mean age of recipients were $45.2{\pm}10.7$ years(range, $25{\sim}59$). Early complications were bleeding, reperfusion injury, and infection and late complications were mainly infection and post-transplantation lymphoproliferative disease. Excluding the operative mortality, the mean survival period was 16.5 months($2{\sim}60$ months). Two retransplantations had been performed 2 weeks and 13 months after single lung transplantations. Conclusion: In order to achieve long term survival, early detection of complications and proper treatment in addition to surgical skills are necessary, and these efforts can promote better lung transplantation programs in the near future.