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Centro de Investigaciones Regionales Dr. Hideyo Noguchi, Universidad Autónoma de Yucatán

2020, Number 3

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Rev Biomed 2020; 31 (3)

Pulmonary lymphangioleiomyomatosis. Do not forget the importance of the pneumothorax as an initial expression

López-Romero S, Moshe Barrera-Pérez HA, Gary K, Vargas-Mendoza GK, Cortés-Tellés A

Language: Spanish
References: 14
Page: 144-148
PDF size: 405.03 Kb.


ABSTRACT

Pulmonary lymphangioleiomyomatosis (PL) is a diffuse cystic lung disease with a low prevalence that affects women between the 3rd and 5th decade of life. Common clinical symptoms are progressive dyspnea, recurrent spontaneous pneumothorax (SPN) and chylothorax. The diagnosis of PL is unfrequently achieved during the diagnostic approach of a recurrent SPN due to its low rate of presentation as an initial expression of the disease.
Here we report a case of a woman of the 5th decade of life with a clinical background of recurrent SPN and progressive dyspnea during the past 4 years, she underwent multiple surgical interventions (placement of pleural tubes) as management of recurrent SPN, however, without a clinical diagnosis. She was referred to our hospital with a clinical diagnosis of bronchopleural fistula. A high-resolution computed tomography (HRCT) scan of the chest was performed during initial evaluation, in which multiple heterogeneous cystic images were observed in the lung parenchyma, also, a bilateral pneumothorax was confirmed. Histopathological analysis of lung tissue obtained by videoassisted thoracic surgery (VATS) confirmed pulmonary PL.


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