title: Gorham-Stout syndrome affecting the temporal bone with cerebrospinal fluid leakage.
abstract: Gorham-Stout syndrome is a rare disorder characterized by progressive osteolysis that leads to the disappearance of bone. Lymphvascular proliferation causes the local destruction of bony tissue. Owing to the low incidence of this syndrome, little is known about its etiology or treatment. We present an 11-year-old girl with Gorham-Stout syndrome that involved right petrous apex in temporal bone and upper clivus, which cause intracranial pressure increase and cerebrospinal fluid (CSF) leakage. The patient required surgical repair of CSF leakage by extradural middle fossa approach with temporal fascia flap. Combined treatment with interferon and propranolol prevented the progression of osteolysis.
authors.affiliation: Department of Otolaryngology, National Center for Child Health and Development, Tokyo, Japan. morimoto-n@ncchd.go.jp
authors.forename: Noriko
authors.initials: N
authors.lastname: Morimoto
authors.forename: Hideki
authors.initials: H
authors.lastname: Ogiwara
authors.forename: Osamu
authors.initials: O
authors.lastname: Miyazaki
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kitamuara
authors.forename: Sachiko
authors.initials: S
authors.lastname: Nishina
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Takanobu
authors.initials: T
authors.lastname: Maekawa
authors.forename: Nobuhito
authors.initials: N
authors.lastname: Morota
mesh_terms: D002559:Cerebrospinal Fluid Rhinorrhea / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging / Q000379:methods; D010015:Osteolysis, Essential / Q000175:diagnosis* / Q000628:therapy*; D013506:Otologic Surgical Procedures / Q000379:methods; D010579:Petrous Bone / Q000473:pathology* / Q000601:surgery; D011433:Propranolol / Q000627:therapeutic use; D035583:Rare Diseases; D011994:Recombinant Proteins / Q000627:therapeutic use; D018570:Risk Assessment; D013524:Surgical Flaps / Q000098:blood supply*; D013577:Syndrome; D013701:Temporal Bone / Q000473:pathology / Q000601:surgery; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D011433:Propranolol
keywords: CSF leak; Interferon-α2; Lymphovascular proliferation; Petrous apex; Proplanolol; VEGF