Open AccessLymphangioleiomyomatosis Unusual Cause of Spontaneous Pneumothorax
Author(s) -
Lamya Al Aamri,
Raafat Dahrouj,
Nasser Al Awaid
Publication year - 2021
Publication title -
international journal of health sciences and research
Language(s) - English
Resource type - Journals
ISSN - 2249-9571
DOI - 10.52403/ijhsr.20210635
Subject(s) - lymphangioleiomyomatosis , medicine , tuberous sclerosis , pneumothorax , lung biopsy , lung , rare disease , histopathology , biopsy , radiology , video assisted thoracoscopic surgery , surgery , disease , pathology
Lymphangioleiomyomatosis (LAM) is a rare multisystem disease, predominately affect premenopausal female. LAM could be an inherited disease associated with Tuberous Sclerosis Complex syndrome or sporadic. Most common pulmonary symptoms are dyspnea and pneumothorax. We report 31- year female, presented with right-side pneumothorax chest drainage was inserted. Further investigation revealed multiple cystic lesions in chest computed tomography images suggestive of LAM disease. She underwent video-assisted thoracoscopic surgery (VATs) to obtain a lung biopsy. Histopathology lung tissue confirms pulmonary Lymphangioleiomyomatosis.Key words: Lymphangioleiomyomatosis; Tuberous Sclerosis Complex syndrome; video-assisted thoracoscopic surgery.