title: Cerebrospinal fluid leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine.
abstract: Patients with Gorham-Stout disease (GSD), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. Destruction of bone matrix is caused by lymphatic vessels, which can lead to CSF leakage if parts of bony structures adjacent to CSF spaces are involved. So far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with CSF leakage. The authors report the case of a 30-year-old man with GSD and CSF leakage due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine. Neurosurgical intervention, including dura repair, was needed. Experimental medical therapy with rapamycin was started, leading to disease control for more than 12 months. Progression of GSD can lead to destruction of the meninges, causing CSF leakage. The authors review 4 other cases reported in the literature and discuss therapeutic options.
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Eric Jose
authors.initials: EJ
authors.lastname: Suero Molina
authors.forename: Thomas
authors.initials: T
authors.lastname: Niederstadt
authors.forename: Vincent
authors.initials: V
authors.lastname: Ruland
authors.forename: Gian
authors.initials: G
authors.lastname: Kayser
authors.forename: Walter
authors.initials: W
authors.lastname: Stummer
authors.forename: Christian
authors.initials: C
authors.lastname: Ewelt
authors.forename: Jochen
authors.initials: J
authors.lastname: Rössler
mesh_terms: D000328:Adult; D065634:Cerebrospinal Fluid Leak / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D018450:Disease Progression; D004388:Dura Mater / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010015:Osteolysis, Essential / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed
keywords: CSF leakage; GSD = Gorham-Stout disease; Gorham-Stout disease; IFN = interferon; osteolytic lesion; thoracic spine