Document Detail


Proteus syndrome: a newly recognized hamartomatous syndrome with significant craniofacial dysmorphology.
MedLine Citation:
PMID:  8601022     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A new disorder describing multiple hamartomas distinct from neurofibromatosis and Klippel-Trenaunay-Weber syndrome was first reported in 1979. It was named Proteus syndrome after the Greek god Proteus, the polymorphous, who could change his shape at will to avoid capture. The clinical manifestations are extensive, including cranial exostoses; progressive enlargement, asymmetry and disfigurement of the skull; macrocephaly; exostoses of the ear canals, nasal bridge, and alveolar ridge; partial gigantism of the hands or feet, asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, and long bone overgrowth. A case report of Proteus syndrome is presented and discussed along with a review of the pertinent literature.
Authors:
A G Tattelbaum; C R Dufresne
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  6     ISSN:  1049-2275     ISO Abbreviation:  J Craniofac Surg     Publication Date:  1995 Mar 
Date Detail:
Created Date:  1996-05-09     Completed Date:  1996-05-09     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  151-60     Citation Subset:  D    
Affiliation:
Department of Plastic Surgery, Georgetown University Medical Center, Washington, DC 20037, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Dental Care for Chronically Ill*
Facial Asymmetry / pathology
Facial Bones / abnormalities*
Female
Humans
Proteus Syndrome* / diagnosis,  pathology,  surgery

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