• Journal of Chest Surgery
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• v.37 no.12
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• pp.1029-1031
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• 2004

We had expierienced pulmonary lymphangioleiomyomatosis(LAM) with bilateral chylothorax and chylous ascites. A twenty-one-year-old lass with chief complaint of abdominal pain was admitted through the emergency room. She received emergent pelvicoscopic surgery for the rupture of the right corpus luteum. We aspirated 1000ml of the uncoagulated blood. The bleeding point was cautherized electrically. LAM was diagnosed with tissue from the retroperitoneum. Chylous ascites and bilateral chylothorax were occurred despite of various treatments. On thoracotomy, bullous changed lung and lymphatic leakage from visceral and parietal pleura were observed. She died of respiratory insufficiency and general weakness after 6 months from admission.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.519-531
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• 1993

Background: Lymphangioleiomyomatosis(LAM) is a very rare disease occurring in women of reproductive age and leading to progressive respiratory failure despite therapy. But the natural history of this disease is uncertain and although anti-estrogenic agents have been used for more than twenty years, it's efficacy is still in debate. This study was performed to enhance understanding of this fatal disease in Korea by examining clinical, radiological, and pathologic findings of all the previously reported cases of LAM on Korea along with four new cases of LAM whom we report in this paper. Method: Out of twlve cases of LAM previously unpublished and published in domestic papers, two cases whose diagnoses were considered doubtful after review of clinical, radiological, and pathologic findings at "Asian Congress on Lymphangioleiomyomatosis" at Kyoto, Japan in feburary of 1993 were excluded from this study. Six cases which were reported previously and four new cases of LAM whom we report in this paper were analysed for the clinical, radiological, and pathologic characteristics. Results: All ten patients were women with mean age of $33{\pm}7$. The most common symptom was exertional dyspnea and most patients had history of pneumothoraces. Pulmonary function tests showed decreased diffusing capacity. on high resolution computed tomography(HRCT), all the cases had characteristic cysts. Most of the patients did not respond to hormonal therapy. Conclusion: In women of reproductive age, presenting with dyspnea who has a history of pneumothorax, LAM should always be considered as one of the diagnostic possibilities. If suspected, HRCT should be done to look for characteristic cysts and if needed, open lung biopsy should be done to confirm the diagnosis.

• Tuberculosis and Respiratory Diseases
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• v.48 no.5
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• pp.788-793
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• 2000

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involved by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.600-604
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• 1995

Lymphangioleiomyomatosis, a rare disease in women of childbearing age, is the result of benign nodular hypertrophy of the smooth muscle of the lypmhatics and other tissues of the abdomen and thorax. We report a 36-years-old woman with pulmonary and retroperitoneal lymphangioleiomyomat.osis who responded with hormone treatment. She developed vaginal pruritis and a pelvic ultraound was done given her significant past medical history. Ultrasound examination demonstrated a large mass in the right side of her pelvis. Therefore she was admitted to St. Michael's Hospital in Toronto for laparoscopy. Result of cytology was to be consistent with the diagnosis of retroperitoneal lymphangioleiomyomatosis. High resolution CT sacn of the thorax demonstrated multiple small cystic lesions, without associated nodularity compatible with a diagnosis of pulmonary lymphangioleiomyomatosis. She has been taking Provera tablets 100mg po tid since Dec. 15, 1993. We have given her a prescription for Depo provera 500mg IM monthly since she came back to Korea. and made arrangements for regular follow up monthly. We performed chest X-ray, CT of chest(high resolution), abdomen and pelvis, pulmonary function tests and arterial blood gas analysis. Chest X-ray and CT findings showed no significant change since July. 20, 1993.

• 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.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.63 no.3
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• pp.289-293
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• 2007

Lymphangioleiomyomatosis (LAM) is a rare disease that affects females of reproductive age. It is characterized by the abnormal proliferation of smooth muscle cells in the lung and along the axial lymphatics. We report a case of lymphangioleiomyomatosis presenting as a lung mass. The patient visited the emergency room because of dyspnea upon exertion. The chest X-ray showed a lung mass in the right lower lung field and a pneumothorax in the left lung. Chest computed tomography revealed a $5{\times}3cm$ sized mass in the right lower lobe and multiple thin-walled small cysts scattered in both lungs. Transbronchial biopsy of the lung mass was performed. The biopsy specimen showed atypical smooth muscle cell proliferation and cystic dilatation of the terminal bronchioles, which confirmed the diagnosis of lymphangioleiomyomatosis. To the best of our knowledge, this is the first case of lymphangioleiomyomatosis presenting as a lung mass.

• Journal of Chest Surgery
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• v.33 no.1
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• pp.88-95
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• 2000

Pulmonary lymphangioleiomyomatosis is a chronic destruct8ive disease of the lung affecting women of childbearing ages which eventually leads to respiratory failure. Lung transplantation is the only conclusive therapeutic measure because this disease responds poorly to other therapies, To date only a few reports in the literature describes the clinical experience of the bilateral sequential lung transplantation of this rare condition. We performed a bilateral sequential lung transplantation on a 32-year-old woman suffering from lymphangioleiomyo-matosisw. The heart-lung block was harvested from a 51-year-old donor. We transplanted the left lung first through the clam-shell incision. As the hemodynamics deteriorated suddenly during the dissection of the right lung the right lung was transplanted under the cardio-pulmonary bypass. Although the patient's lung function was initially satisfactory the patient died of sepsis and subsequent cardiogenic shock at the postoperative 18th day. Autopsy findings showed infection of Candida albicans on the pericardium and the left lung which had been initiated possibly from the left bronchial anastomosis site,. Through detailed review of the clinical course we concluded that lung transplantation could have been performed safely on this disease provided that early diagnosis and proper management or the oppor-tunistic infection have been carried out.

• Journal of Yeungnam Medical Science
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• v.27 no.1
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• pp.63-68
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• 2010

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutation in the tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread and a remarkable female gender predilection. The pathology of LAM is represented by the proliferation of immature smooth muscle cells in the walls of airways, and venules and lymphatic vessels in the lung. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax and collections of chylous fluid. The diagnosis of pulmonary LAM can be made on chest X-ray, a high-resolution CT scan and lung biopsy. We experienced a case of pulmonary lymphangioleiomyomatosis in a 28-years-old female patient who had suffered from progressive dyspnea on exertion, so we report on it along with a brief review of the relevant literature.