Anesthetic Management in Birt-Hogg-Dubé Syndrome with Spontaneous and Recurrent Pneumothorax during Recovery after Laparoscopic-Assisted Partial Nephrectomy

Abstract

Birt-Hogg-Dubé Syndrome (BHDS) is an inherited autosomal dominant disorder associated with 3 clinical manifestations, including skin fibrofolliculomas, multiple lung cysts and kidney tumors. These symptoms generally do not appear till adult. BHDS can be suggested by clinical manifestations but is definitively confirmed by molecular genetic testing to detect mutations in the FLCN gene [1][2][3][4].The pulmonary manifestations of BHDS are common findings and mainly related to multiple lung cysts and development of spontaneous pneumothorax. In the rate of occurrence of pneumothorax, patients