title: [Dyspnea caused by bullae in a muscular dystrophy patient on chronic intermittent ventilatory support].
abstract: A 65-year-old patient, ex-smoker, with facioscapulohumeral muscular dystrophy (FSHD) had been on home non invasive ventilatory support for three years when he experienced gradual increase of dyspnoea. The chest radiograph showed large bullae occupying most of the right hemithorax, with compression of lung tissue, mediastinal shift, and compression of the left lower lobe. Bullectomy resulted in rapid clinical and radiographic improvement. This is the first report of beneficial effects of emergency bullectomy in FSHD. Bullectomy has proved most successful in patients with localized bullae and compression of surrounding lung tissue. Patients with respiratory infections and bronchiectasis benefit less.
authors.affiliation: Afd. Anesthesiologie, Academisch Ziekenhuis, Groningen.
authors.forename: G
authors.initials: G
authors.lastname: van Santen
authors.forename: J J
authors.initials: JJ
authors.lastname: Meuzelaar
authors.forename: J E
authors.initials: JE
authors.lastname: Tulleken
authors.forename: J G
authors.initials: JG
authors.lastname: Zijlstra
authors.forename: A F
authors.initials: AF
authors.lastname: Meinesz
authors.forename: T S
authors.initials: TS
authors.lastname: van der Werf
mesh_terms: D000368:Aged; D004417:Dyspnea / Q000209:etiology*; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation / Q000009:adverse effects; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D008297:Male; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000150:complications*; D011656:Pulmonary Emphysema / Q000150:complications* / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D013510:Pulmonary Surgical Procedures / Q000379:methods; D011859:Radiography; D012907:Smoking / Q000009:adverse effects; D016896:Treatment Outcome