Cystic lung disease in birt-hogg-dubé syndrome: a case series of three patients

Abstract

Birt-Hogg-Dubé syndrome is characterized by clinical manifestations such as hamartomas of the skin, renal tumors and lung cysts with spontaneous pneumothoraces. Patients with Birt-Hogg-Dubé syndrome may present with only multiple lung cysts. We report the chest computerized tomography (CT) features of three patients with Birt-Hogg-Dubé syndrome. Each patient had multiple lung cysts of various sizes according to chest CT evaluation, most of which were located in lower lobes and related to pleura. The identification of unique characteristics in the chest CT of patients with Birt-Hogg-Dubé syndrome may provide an efficient mechanism for diagnosis.

Keywords: Birt-Hogg-Dubé syndrome; chest CT; lung cyst.

Figures

Figure 1.

Coronal reformatted CT scan of Patient 1 demonstrates a limited pneumothorax in the right lung; also note multiple bilateral lung cysts. CT: computed tomography

Figure 2.

a, b.Coronal and axial CT images of Patient 2 show multiple bilateral lung cysts (a). The largest cyst in the right lung is related to pleura and in the middle lobe (b). CT: computed tomography