title: Spontaneous bilateral chylothorax with fatal outcome in a patient with melorheostosis.
abstract: We report a case of progressive, multifocal melorheostosis in a 28-year-old woman, with involvement of the left arm, chest, spine, and impressive soft tissue involvement. In the past, she had undergone multiple vascular interventions. She presented with spontaneous massive bilateral chylothorax. After conservative treatment without success, we conducted bilateral pleurodesis. This resulted in a clear reduction of pleural effusions, but her medical condition subsequently worsened due to progressive parenchymatous infiltrates, and increased interlobal pleural effusions. She ultimately died of global respiratory insufficiency. In patients with melorheostosis, involvement of the soft tissue can result in distinctive morbidity, and whenever possible, treatment should be conservative.
authors.affiliation: Division of Endocrinology, Diabetes and Clinical Nutrition, University Hospital, Berne, Switzerland.
authors.forename: Michèle
authors.initials: M
authors.lastname: Leuenberger
authors.forename: Jan
authors.initials: J
authors.lastname: Braunwalder
authors.forename: Ralph A
authors.initials: RA
authors.lastname: Schmid
authors.forename: Zeno
authors.initials: Z
authors.lastname: Stanga
mesh_terms: D000328:Adult; D002916:Chylothorax / Q000150:complications* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008557:Melorheostosis / Q000150:complications* / Q000503:physiopathology; D010289:Parenteral Nutrition, Total; D018700:Pleurodesis; D012131:Respiratory Insufficiency