Pľúcna forma lymfangioleiomyomatózy komplikovaná výskytom pneumotoraxu u mladého pacienta – kazuistika // SOLEN

Slovenská chirurgia 3-4/2016

Pulmonary lymfangioleiomyomatosis complicated with pneumotorax described in case of a young patient – case report

Lymfangioleiomyomatosis is a rare congenital disease that almost exclusively affects women in a reproductive age. There is a close connection between lymfangioleiomyomatosis and tuberous sclerosis. Pulmonary LAM in men is extremely rare. Because of mutation in TSC genes, neoplastic smooth muscle cells from an unknown source metastasize to the lung and drive destructive remodeling of lungs, which leads to formation of thin-walled cystic lesions. We describe the case of a young patient with LAM and secondary spontaneous pnemotorax hospitalised at the Second Department of Surgery, Pavol Jozef Šafarik University, Faculty of Medicine and Louis Pasteur University Hospital Košice.

Keywords: lymfangioleiomyomatosis, pneumotorax