Document Detail

Wyburn-Mason syndrome: a case report.
MedLine Citation:
PMID:  20681369     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Wyburn-Mason syndrome is a rare disease associated with multiple arteriovenous malformations of the brain, orbit, and face resulting from an insult occurring during embryonic development. CASE REPORT: We present the clinical and radiological features of a 47-year-old-man with Wyburn-Mason syndrome who suffered from recurrent bleeding episodes primarily at the nasal corner of the left orbit. After radiotherapy and several angioembolisations, surgical reduction with exenteration of the left orbit was performed and resulted in reduced bleeding. Two years later the patient presented with maxillary sinus empyema. Due to massive endonasal bleeding, endoscopy could not be performed and the maxillary empyema was treated via a transorbital approach. CONCLUSION: Although close observation represents the standard of care in Wyburn-Mason syndrome, patient-specific management decisions are required in the presence of symptoms or complications. In the presented case, surgical intervention proved to be successful.
S Wiegand; B Eivazi; S Bien; A M Sesterhenn; A Teymoortash; J A Werner
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  B-ENT     Volume:  6     ISSN:  1781-782X     ISO Abbreviation:  B-ENT     Publication Date:  2010  
Date Detail:
Created Date:  2010-08-04     Completed Date:  2010-09-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101247842     Medline TA:  B-ENT     Country:  Belgium    
Other Details:
Languages:  eng     Pagination:  139-41     Citation Subset:  IM    
Department of Otolaryngology, Head and Neck Surgery, University Hospital Giessen, & Marburg, Campus Marburg, Germany.
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MeSH Terms
Arteriovenous Malformations / complications*,  therapy
Embolization, Therapeutic / methods
Empyema / radiography,  surgery
Fatal Outcome
Hemorrhage / radiotherapy
Intracranial Arteriovenous Malformations / complications,  diagnosis,  therapy
Magnetic Resonance Imaging
Maxillary Sinus / radiography,  surgery
Middle Aged
Orbit Evisceration
Orbital Diseases
Paranasal Sinus Diseases / radiography,  surgery

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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