Lung transplantation for pulmonary lymphangioleiomyomatosis:a report of 9 cases / 中华胸心血管外科杂志

Objective To investigate the selection of recipients,operative technique, and perioperative management of lung transplantation.Methods From June 2013 to September 2016, 9 patients with pulmonary lymphangioleiomyomatosis un-derwent lung transplantation at Affiliated Wuxi People's Hospital.There were 9 females aged from 23 to 52 years.Chest en-hancement of CT in 9 patients prior to transplantation suggests multiple different sized thin-walled cystic shadows in whole lung. Nine patients received bilateral sequence lung transplantation ( BSLT) .The pathological findings after the operation indicated that cells of the abnormal proliferation of smooth muscle diffused around the bronchi , vessels and lymphatics , and widened the alveolar septum, which was consistent with the pathological changes in the patients with PLAM .We analyzed the differences of oxygenation index, forcedexpiratory volume at 1 sec(FEV1), and DLCO％ pred.Using paired t test to compare these indica-tors, P<0.05 was considered statistically significant.Results The oxygenation index was increased from(210 ±55) mmHg (1 mmHg=0.133 kPa) to(400 ±80)mmHg after treatment, the difference was statistically significant(P<0.05).The FEV1 was increased from(1.0 ±0.2)L to(2.8 ±0.4)L, and the difference was statistically significant(P<0.05).The DLCO％pred was increased from(27.5 ±2.5)％ to(75.0 ±10.0)％, and the difference was statistically significant(P<0.05).Fi-nally, 8 patients were discharged from the hospital, and the pulmonary function was good.1 patients died of infection and acute rejection on the 13 day after operation.Conclusion Lung transplantation is effective in the treatment of chronic thromboem-bolic pulmonary hypertension , and long-term survival requires further investigations .Lung transplantation is the only effective means for the treatment of end-stage pulmonary lymphangioleiomyomatosis .Bilateral sequence lung transplantation is the first choice.

Linfagioleomiomatose pulmonar em paciente pediátrico: relato de caso / Pulmonar lymphagioleiomatosis in a pediatric patient: a case report

A linfangioleiomiomatose pulmonar (LAM) é considerada uma doença rara, com predileção de acometimento em mulheres durante período reprodutivo, principalmente entre a terceira e quarta década de vida. A doença é atualmente reconhecida como uma neoplasia de baixo grau e se caracteriza por proliferação de células musculares lisas atípicas no parênquima pulmonar. Dispneia, pneumotórax recorrente e tosse seca são as manifestações clínicas mais comuns. Em exames de imagem, é notada comumente a existência de hiperinsuflação, infiltrado intersticial reticulonodular e cistos de diferentes tamanhos em topografia pulmonar. A presença concomitante de angiomiolipomas pode ser verificada em alguns pacientes. Tais achados radiológicos, quando associados ao quadro clínico característico , falam a favor do diagnóstico de LAM, sendo possível a confirmação pela histopatologia. A despeito das diferentes formas de tratamento existentes para a LAM, ainda não existe medida curativa para a doença. Em geral, os pacientes evoluem com contínua deterioração da função pulmonar, com predomínio de um distúrbio ventilatório obstrutivo que deve ser diferenciado de outras afecções mais prevalentes, como asma e doença pulmonar obstrutiva crônica. Neste trabalho, relata-se um caso de paciente em idade pediátrica, com presença de achados clínicos e radiológicos compatíveis com o diagnóstico de LAM, ainda que fora da faixa etária usual de acometimento da doença.

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease which affects women during reproductive age mainly between thirdies and forties. Currently the disease is recognized with a low grade neoplasm and it is characterized by proliferation of atypical smooth muscle cells in the lung parenchyma. Dyspnea, recurrent pneumothorax and dry cough are the most common clinical manifestations. On imaging studies, the presence of hyperinsuflation, interstitial infiltrates reticulonodular and cysts of different sizes in lung topography is common. The concomitant presence of angiomyolipoma can be recorded in some patients. When associated with the usual clinical condition, the radiological findings suggest the diagnosis of LAM and it can be confirmed by histopathology. Despite of different forms of treatment for LAM, there is still no curative method for it. In general, the patients develop continuous deterioration of lung function with a predominance of obstructive lung disorder which must be distinguished from other more prevalent diseases, such as asthma and chronic obstructive pulmonary disease. In this paper, we report a case of a child with the presence of clinical and radiological findings compatible with the diagnosis of LAM although it is outside the usual age of onset of the disease.

CT features of pulmonary lymphangioleiomyomatosis / 实用放射学杂志

Objective To explore the CT features of pulmonary lymphangiomyomatosis (PLAM).Methods Clinical and high resolution CT (HRCT)data of 14 patients with pathologically proved PLAM were analyzed retrospectively.The clinical and CT features were summarized by combining the literatures.Results All 14 cases were female.They all presented with dyspnea in different degree after the activity. Scattered or widely distributed translucent and cystic lesions with indistinct walls in bilateral lungs were seen on routine CT images. HRCT showed homogeneous clear thin-walled cysts with diameter ranging from several millimeters to 25 mm,wall thickness of 1-2 mm,and surrounded by normal lung tissue.Meanwhile,blood vessels were found around the cysts,and there were no central lobular cores.The cysts were different sizes and irregular distribution.6 patients had extra-pulmonary CT manifestations:1 case with intracranial multiple sclerosis, hepatic and renal angiomyolipomas,and hepatic multiple hemangiomas,3 cases with mediastinal,hepatic and renal angiomyolipomas, and 2 cases with retroperitonea lymphangioleiomyomatosis.Conclusion The CT of PLAM is characterized by the diffuse distribution of thin-walled cystic cavities and the wall thickness is generally uniform.The typical manifestations of HRCT combined with clinical data have great values in the early diagnosis and differential diagnosis.

A Case of Pregnancy in Patient with Pulmonary Lymphangioleiomyomatosis / 대한산부인과학회잡지

Pulmonary lymphangioleiomyomatosis is a rare disorder of unknown cause and characterised by hamartomatous proliferation of smooth muscle occurring in women of reproductive age exclusively. It causes dyspnea, recurrent pneumothorax, chylothorax, hemoptysis and respiratory failure eventually. Chest radiographs show diffuse interstitial infiltrates and cysts of uniform size, and pulmonary function tests often show airflow limitation with increase in residual volume. Hormonal factors are thought to play a role because it generally affects premenopausal women, but there is no definite treatment yet. We present an unusual case of pulmonary lymphangioleiomyomatosis during normal pregnancy with a review of literature.

Pulmonary lymphangioleiomyomatosis in Pham Ngoc Thach Hospital: a case report

This rare condition was diagnosed in the stage of a history of resection of left renal tumor of angiomyolipoma and recurrant pneumothrax with PCR of positive tuberculosis bacilla, special chest CT scan with thin wall cyst. These female patients are in reproductive age, using consistantly sex hormone. The prognosis is similar with COPD. Early detection and treatment of the case slowers the progression of disease.

A Case of Lymphangioleiomyomatosis with Renal Angiomyolipoma / 결핵

Recently we have experienced one case of pulmonary lymphangioleiomymatosis(LAM). A 49 year-old woman visited the outpatient department complaining of longstanding dyspnea, which was aggravated by exercise. Although the chest PA film showed nothing more than a slight increase in interstitial marking, a lung HRCT revealed multiple cystic lesions of a similar size that were scattered through out the whole field in both lungs. An abdominal CT detected an angiomyolipoma located in the midbody of the left kidney. Video-assisted thoracic surgery(VATS) was performed for the pathologic diagnosis. On gross examination of the biopsy lung, a pulmonary LAM was confirmed by a finding of smooth muscle proliferation in the interstitum of the lung. After the final diagnosis, oral medroxyprogesterone was prescribed and she is presently in a stable condition.

A Case of Pulmonary Lymphangioleiomyomatosjs Associated with Thberous Sclerosis and Renal Angiomyolipoma / 결핵

Lymphangioleiomyomatosis(LAM) is rare and essentially limited to women in the reproductive ages. A 39-year-old female was admitted due to progressive exerUional dyspnea and intermittent productive cough. Chest PA showed marked hyperinflation of the lung associated with a diffuse reticulo-nodular pattern. High resolution CT scan of the thorax demonstrated that diffusely scattered thin-walled cysts were distributed throughout the bilateral lung fields. Abdominal CT scan showed variable sized multiple angiomyolipoma of both kidney. By open lung biopsy, she was diagnosed as pulmonary LAM associated with Tuberous sclerosis and renal Angiomyolipoma. We present the case and discuss the connection between pulmonary LAM and Tuberous sclerosis.

A Case of Lymphangioleiomyomatosis Looked Like Miliary Tuberculosis / 결핵

We experienced one case of pulmonary lymphangioleiomyomatosis in 26-year-old female patient. She had taken antituberculous medication under the impression of miliary tuberculosis on simple chest X-ray at peripartum period. On outpatient follow-up she complained of progressive exertional dyspnea in spite of medication. Through careful history taking and physical examination, high resolutional CT, and open lung biopsy she was diagnosed as pulmonary lymphangioleiomyomatosis combined with incomplete type of tuberous sclerosis. So, we presented the case with the brief review the literatures.