| Gorham-stout syndrome of the petrous apex causing chronic cerebrospinal fluid leak. | |
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MedLine Citation:
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PMID: 20502378 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To describe the clinical course, diagnostic features, and treatment of a case of Gorham-Stout syndrome involving the petrous apex and causing chronic cerebrospinal fluid (CSF) leak. STUDY DESIGN: Clinical capsule report. SETTING: Academic pediatric hospital. PATIENT: A 12-year-old boy presented with a destructive lesion of the right petrous apex and a 1-month history of headache, nausea, and vomiting. INTERVENTIONS: Computed tomographic and magnetic resonance imaging revealed a nonenhancing lytic lesion of the right petrous apex and mandibular condyle. The lesions were bright on T2-weighted imaging with high signal intensity extending into the surrounding soft tissues. Leptomeningeal enhancement and an opening pressure of 0 cm of water on lumbar puncture suggested chronic CSF hypotension. Initial surgical exploration revealed diffuse infiltration of CSF into the soft tissue lateral to the temporal bone. Subsequent middle ear and mastoid obliteration was performed to definitely repair the CSF leak. RESULTS: The clinical presentation, physical, laboratory, radiologic, and operative findings are consistent with a diagnosis of Gorham-Stout syndrome. In this case, lymphangiomatosis led to massive osteolysis of the petrous apex with CSF fistula into the surrounding soft tissues and middle ear with chronic intracranial hypotension. CONCLUSION: This is the second report of chronic CSF leak resulting from lymphangiomatosis of the cranial base (Gorham-Stout syndrome). |
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Authors:
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Sharon L Cushing; Gisele Ishak; Jonathan A Perkins; Jay T Rubinstein |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology Volume: 31 ISSN: 1537-4505 ISO Abbreviation: Otol. Neurotol. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-06-28 Completed Date: 2010-10-08 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100961504 Medline TA: Otol Neurotol Country: United States |
Other Details:
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Languages: eng Pagination: 789-92 Citation Subset: IM |
Affiliation:
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Division of Pediatric Otolaryngology Head and Neck Surgery, Seattle Children's Hospital, University of Washington, Seattle, Washington, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Biopsy Child Humans Lymphangioma / cerebrospinal fluid, pathology Magnetic Resonance Imaging Male Osteolysis, Essential / cerebrospinal fluid*, pathology*, surgery Otologic Surgical Procedures Petrous Bone / pathology*, surgery Skull Neoplasms / cerebrospinal fluid, pathology Tomography, X-Ray Computed |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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