Abstract
: Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant genetic disorder caused by mutations in the folliculin gene (FLCN). The three clinical manifestations of BHDS include benign skin tumors, renal tumors, and multiple pulmonary cysts and result in a refractory pneumothorax. Because the pulmonary cysts in BHDS are numerous and widely dispersed, it is not feasible to resect all of the cysts. We recently reported total pleural covering (TPC) via 4-port video-assisted thoracic surgery (4P-VATS) that covered the entire visceral pleura with oxidized regenerated cellulose (ORC) mesh. Moreover, 4P-VATS TPC successfully prevented the recurrence of pneumothoraces in patients with BHDS. As a minimally invasive procedure, we report a case of secondary pneumothorax in a patient with BHDS who was successfully treated by uniportal VATS (U-VATS) TPC with ORC mesh.
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