Catalog
Lymphangioleiomyomatosis associated with tuberous sclerosis complex
Link to WHO Definition
Lymphangioleiomyomatosis associated with tuberous sclerosis complex – http://id.who.int/icd/entity/1746892004
This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.