title: Total pleural covering technique for intractable pneumothorax in patient with Ehlers-Danlos syndrome.
abstract: We report a patient with vascular-type Ehlers-Danlos syndrome (vEDS) who developed pneumothorax and was treated with a total pleural covering technique (TPC). A 24-year-old man developed repeat pneumothorax with intermittent hemo-sputum. Based on unusual radiological manifestations of lung lesions and physical findings, EDS was suspected as an underlying cause of the pneumothorax. Surgical treatment was performed using a mediastinal fat pad and TPC, and no relapse was seen up to 2 years after surgery. TPC is a less invasive surgical approach for selected patients with vEDS. Accurate underlying diagnosis of vEDS and systemic evaluation of vascular complications are necessary before planning surgery.
authors.affiliation: Department of General Thoracic Surgery, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, 3-7-1 Habikino, Habikino, Osaka, Japan. kadotay@ra.opho.jp.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Kadota
authors.affiliation: Department of General Thoracic Surgery, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, 3-7-1 Habikino, Habikino, Osaka, Japan.
authors.forename: Eriko
authors.initials: E
authors.lastname: Fukui
authors.affiliation: Department of General Thoracic Surgery, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, 3-7-1 Habikino, Habikino, Osaka, Japan.
authors.forename: Naoto
authors.initials: N
authors.lastname: Kitahara
authors.affiliation: Department of General Thoracic Surgery, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, 3-7-1 Habikino, Habikino, Osaka, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Okura
authors.affiliation: Department of General Thoracic Surgery, Osaka Prefectural Medical Center for Respiratory and Allergic Diseases, 3-7-1 Habikino, Habikino, Osaka, Japan.
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Ohta
mesh_terms: D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D010994:Pleura / Q000601:surgery*; D011030:Pneumothorax / Q000209:etiology / Q000601:surgery*; D012008:Recurrence; D055815:Young Adult
keywords: Ehlers–Danlos syndrome; Pneumothorax; Total pleural covering technique