[Secondary spontaneous pneumothorax in tuberous sclerosis].
Secondary pneumothorax occurs as a symptom of an underlying pulmonary disease. We report the case of an 18-year-old woman with tuberous sclerosis (Bourneville's disease) and recurrent pneumothoraces. Clinical outcome was favorable 6 months after bilateral videothoracoscopic pleurectomy. The complete triad of tuberous sclerosis (TS) (mental retardation, seizures, adenoma sebacium) is not always present in those who develop pulmonary involvement. When TS involves the lung it is clinically and pathologically indistinguishable from lymphangioleiomyomatosis (LAM). The very rare pulmonary involvement of TS and LAM are problems primarily of women in childbearing age. A pneumothorax can be the first symptom of TS or LAM.
Kyriss
T
T
Abteilung für Thoraxchirurgie, Klinik Schillerhöhe, Zentrum für Pneumologie und Thoraxchirurgie, Gerlingen. kyriss@klinik-schillerhoehe.de
Eulenbruch
H
H
Friedel
G
G
Toomes
H
H
Bosse
A
A
D000293:Adolescent; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D011013:Pneumonectomy; D011030:Pneumothorax / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D020775:Thoracic Surgery, Video-Assisted; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*