Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management

Kania, Brooke; Jain, Swapna; West, Brittanie; Courtney, Suzanne W

doi:10.7759/cureus.8386

Cited by 3 publications

(2 citation statements)

References 14 publications

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“…After excluding duplication, we have included published articles in our study, which consists of 33 case reports (Table 2 ) [ 12 - 42 ].…”

Section: Reviewmentioning

confidence: 99%

The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports

Shah,

Patel,

Shah

et al. 2023

Cureus

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Lymphangioleiomyomatosis (LAM) is a rare, slow-growing metastasizing neoplasm in which smooth muscle-like cells infiltrate the lung parenchyma and cause cystic lung damage. The common early symptoms include shortness of breath, pneumothorax, and chest pain. Lymphangioleiomyomatosis mainly involves the lungs, kidneys, and lymph nodes. This study reviews the characteristics of lymphangioleiomyomatosis to identify any possible changes in the prevalence of symptoms of the disease. We conducted a literature review of case reports on lymphangioleiomyomatosis from PubMed and Google Scholar. Variables of interest were age, gender, symptoms, vitals, immunostaining, and radiological findings. Data were transferred to an Excel spreadsheet (Microsoft Corporation, Redmond, WA), and mean, median, standard deviation, frequencies, and proportions were calculated using R version 1.1.456 (RStudio: Integrated Development for R. RStudio, PBC, Boston, MA). Lymphangioleiomyomatosis is a rare case and so not much of the literature could be found online. Thirty-three case reports were included in this study, and females accounted for 78.78% of the presentations. The average age was 38 years, SD 14.41 years. Shortness of breath was the most frequent symptom (60.6%), followed by pneumothorax (57.57%), chest pain (42.42%), cough (24.24%), and pleural effusion (1.25%).

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“…After excluding duplication, we have included published articles in our study, which consists of 33 case reports (Table 2 ) [ 12 - 42 ].…”

Section: Reviewmentioning

confidence: 99%

The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports

Shah,

Patel,

Shah

et al. 2023

Cureus

View full text Add to dashboard Cite

show abstract

“…Lymphangiomyomatosis (LAM) is a rare multisystemic disease characterized by cystic lung lesions, lymphatic abnormalities, and angiomyolipomas [1]. The spectrum of clinical presentations of LAM is variable, but often the rst symptom is recurrent pneumothorax [2].…”

Section: Introductionmentioning

confidence: 99%

Left Uniportal VATS for Lung Decortication After Chemical Pleurodesis Under Spontaneous Breathing in Patient with Lymphangiomyomatosis – A Case Report.

Mantovani

Vannucci

Bassi

et al. 2021

Preprint

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Background: Pneumothorax can be the first symptom of lymphangioleiomyomatosis. Patients with lymphangioleiomyomatosis have a higher risk of recurrence of pneumothorax. Chemical pleurodesis is a viable option to treat the recurrence, but in rare cases, it is not the solution. Case Presentation: We present the case of a patient with lymphangioleiomyomatosis undergoing a talc poudrage via video-assisted thoracoscopic surgery for pneumothorax that failed to re-expand the lung. We proposed to the patient a surgical approach to debride the lung parenchyma with the patient under deep sedation with spontaneous breathing. The patient was discharged on the 5th postoperative day. The chest computed tomography scan showed complete lung re-expansion. Conclusion: We advocate that video-assisted thoracoscopic surgery in patients who are awake is a feasible surgical option that permits the restoration of physiological lung expansion in selected patients who underwent chemical pleurodesis and minimizes the risk of one-lung ventilation.

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The benefit of indwelling pleural catheter with ambulatory pneumothorax device and autologous blood patch pleurodesis in lymphangioleiomyomatosis with persistent air leak

Low

Abeed

et al. 2023

Respirology Case Reports

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We report a 35‐year‐old woman who presented with dyspnoea and chest pain for 1 week. High‐resolution computed tomography (HRCT) thorax revealed bilateral pneumothoraces with diffuse lung cysts. Bilateral intercostal chest tubes were inserted, and there was a persistent air leak (PAL) bilaterally. We performed an autologous blood patch pleurodesis (ABPP) for the left PAL. For the right PAL, she underwent a successful right video‐assisted thoracic (VATS) surgery, wedge biopsy, and surgical pleurodesis. Histopathology examination confirmed the diagnosis of lymphangioleiomyomatosis (LAM). The left pneumothorax recurred. An indwelling pleural catheter (Rocket® IPC™; Rocket Medical plc; WASHINGTON) was inserted and the patient was discharged after 1 day with an atrium pneumostat (Pneumostat™; Atrium Medical Corporation, Hudson, NH, USA) chest drain valve. The patient was initiated on Sirolimus 2 mg daily. The left PAL resolved at 6 weeks. This case highlights the benefit of IPC with an ambulatory pneumothorax device in a patient with LAM with PAL.

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Lymphangioleiomyomatosis: A Case Report and Review of Clinical Features and Management

Cited by 3 publications

References 14 publications

The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports

The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports

Left Uniportal VATS for Lung Decortication After Chemical Pleurodesis Under Spontaneous Breathing in Patient with Lymphangiomyomatosis – A Case Report.

The benefit of indwelling pleural catheter with ambulatory pneumothorax device and autologous blood patch pleurodesis in lymphangioleiomyomatosis with persistent air leak

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