Pleural Disease in Lymphangioleiomyomatosis
Section snippets
Pneumothorax
Pneumothorax is defined as the abnormal presence of air in the pleural cavity. Pneumothorax can occur traumatically by the introduction of ambient air after penetration of the chest wall and pleura or spontaneously. Spontaneous pneumothorax can occur in patients without clinically apparent underlying lung disease (primary spontaneous pneumothorax) or in patients with pulmonary disorders (secondary spontaneous pneumothorax). Secondary spontaneous pneumothorax can occur with virtually any
Chylothorax
Chylothorax is defined as the accumulation of chyle in the pleural space [53]. It results from disruption or obstruction of the thoracic duct or its tributaries in the thorax by tumor, trauma, or surgery, leading to leakage of chyle into the thoracic cavity [53], [54]. Chylothorax also may complicate medical disorders, such as superior vena cava syndrome, lymphatic disorders such as lymphangiomatosis, and yellow nail syndrome [53], [55], [56], [57]. Chylothorax is a well-recognized complication
Summary
LAM is a rare, gender-restricted interstitial lung disease that causes significant morbidity and mortality. Pneumothorax or chylothorax occurs during the course of illness in most patients. The incidence of secondary spontaneous pneumothorax in LAM is among the highest of all chronic lung diseases and may result in considerable morbidity. The effect of pneumothorax and chylothorax on disease progression and prognosis is unknown, however. Most patients require a definitive chemical or surgical
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Cited by (39)
Chylothorax and Pseudochylothorax
2021, Clinics in Chest MedicineCitation Excerpt :This disease is most commonly seen in women, believed to be accelerated by estrogen, and as such typically manifests before menopause around the third to fourth decade of life. Pleural effusion is seen in 20% to 40% of patients with LAM during the course of the disease and is usually chylous.38,41,42 The development of chylothorax in LAM likely results from obstruction or disruption of lymphatic vessels or the thoracic duct by proliferating LAM cells.43–45
Lymphangioleiomyomatosis
2021, Encyclopedia of Respiratory Medicine, Second EditionImaging of cystic lung disease
2015, Seminars in RoentgenologyCitation Excerpt :Chest radiograph findings are often normal or near normal with expanded lung volumes; cystic changes can be occasionally seen (Fig. 1). Pneumothorax is an initial manifestation in 21%-63% of patients with LAM,6 although approximately 66% of patients experience a pneumothorax during the course of their disease.7 At CT, the cysts in LAM are typically smooth and round or oval measuring 2-5 mm diffusely distributed throughout both lungs (Fig. 2).
Daunting complications of one-lung ventilation: Pneumothorax in a patient with lymphangioleiomyomatosis (LAM)
2014, Journal of Clinical AnesthesiaAir travel and pneumothorax
2014, ChestLung transplantation in sporadic lymphangioleiomyomatosis: Study of 7 cases
2013, Medicina Clinica