[Clinical case of the month. A recurrent chylothorax in Bourneville tuberous sclerosis].
Bourneville's disease, first described in 1862, is a phacomatosis that is either autosomal dominant or sporadic. Its typical clinical signs include mental retardation, epilepsy and cutaneous adenomas. The pulmonary form is rare, less than 1%, and is secondary to occlusion of the bronchus, vascular and lymphatics by immature smooth muscle cells. Chylothorax may appear in more than 50% of all cases. No guidelines currently exist for treatment of recurrent chylothorax. However, several possibilities are described in the literature.
Bustin
F
F
Service de Pneumologie, Université de Liège.
Gustin
M
M
Robin
M
M
Cambier
P
P
Warling
X
X
Chantraine
J M
JM
D000328:Adult; D002916:Chylothorax / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D004322:Drainage; D005260:Female; D006801:Humans; D010102:Oxygen Inhalation Therapy; D017410:Practice Guidelines as Topic; D012008:Recurrence; D013907:Thoracostomy; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications* / Q000453:epidemiology / Q000235:genetics