Pleural Covering Application for Recurrent Pneumothorax in a Patient with Birt-Hogg-Dubé Syndrome

Abstract

IntroductionBirt-Hogg-Dubé syndrome (BHDS), a hereditary genodermatosis caused by a germline folliculin (FLCN) mutation, was first described in 1975 and 1977. 1,2) The thoracic manifestations of this syndrome are multiple lung cysts and pneumothorax with frequent recurrences. 3) These cysts appear predominantly at the lower-medial zone of the lung field and adjacent to the interlobar fissure but sometimes abut peripheral pulmonary vessels. 4,5) Thus, the conventional surgical approach in which all subpleural cysts and bullae are resected is not feasible for the management of pneumothorax in BHDS. Here, we describe a patient with BHDS whose recurrent pneumothorax was successfully resolved by applying a pleural covering technique during thoracoscopic surgery. 6) This procedure was effective in preventing further recurrence. Case ReportA 30-year-old male presented at the emergency department of our hospital, complaining of a sudden left-sided chest pain. A chest X-ray and computed tomography showed collapse of the left lung and multiple lung cysts. He was diagnosed with left-sided pneumothorax and treated with the insertion of a portable drainage kit Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary disease that presents with multiple lung cysts and recurrent pneumothorax. These cysts occupy predominantly the lower-medial zone of the lung field adjacent to the interlobar fissure, and some of them abut peripheral pulmonary vessels. For the surgical management of pneumothorax with BHDS, the conventional approach of resecting all subpleural cysts and bullae is not feasible. Thus, after handling several bullae by using a stapler or performing ligation as a standardized treatment, we applied to a pleural covering technique to thicken the affected visceral pleura and then to prevent recurrence of pneumothorax. We herein report the successful application of a pleural covering technique via thoracoscopic surgery to treat the recurrent pneumothorax of a 30-year-old man with BHDS. This technique is promising for the management of intractable pneumothorax secondary to BHDS.Keywords: Birt-Hogg-Dubé syndrome, multiple lung cysts, recurrent pneumothorax, thoracoscopic surgery H, et al. (Thoracic Egg ® , Sumitomo Bakelite CO., Ltd. Tokyo, Japan), that enables to manage him in a setting of outpatient clinic. One-week after insertion of the tube, resolution of the pneumothorax enabled removal of the chest drainage tube. The patient was an ex-smoker (10 packs/ year) but had no medical problems in the past. However, a noteworthy comment was that his father and brother had medical histories of pneumothorax. Since this patient had a family history of pneumothorax and had multiple lung cysts localized predominantly at the lower-medial zone of the lung fields (Fig. 1), a diagnosis of BHDS was strongly suspected. Genetic testing was performed using peripheral blood leucocytes, and then demonstrated that he had an insertion of cytosine (C) in exon 11 of the FLCN gene (c.1285dupC), thereby confirming the diagnosis. Subsequently, afte...

Birt–Hogg–Dubé syndrome: a case report and a review of the literature

“…Thorascopic pleural covering technique has also been suggested as a way of preventing recurrence of pneumothorax in patients with BHDS, but further studies are needed. [ 51 , 52 ]…”

Novel clinical scoring system to identify patients with pneumothorax with suspicion for Birt–Hogg–Dubé syndrome

“…Based on these presentations, the conventional treatment approach in which all subpleural cysts and bullae are resected or subjected to pleuredosis is not feasible for the management of PTX in BHDS. Therefore, it is important for pulmonologists and thoracic surgeons to be aware of this rare cause of PTX and to consider a new therapeutic method such as the total pleural covering technique …”

A Rare Case of Spontaneous Pneumothorax Recurrence 30 Years After Surgery in a Patient with Birt-Hogg-Dube Syndrome: Case Presentation and Short Review of the Literature

“…Treatment approaches for BHDS-related pneumothorax vary, but chemical and surgical pleurodesis have shown potential. Thoracoscopic pleural covering technique is being explored for pneumothorax recurrence prevention ( 14 ). The recurrence rate for BHDS-related pneumothorax is 9.1% and 53.1% after surgery and conservative therapy, respectively, warranting further research on long-term therapeutic effects.…”