Document Detail


Orofacial findings in the Klippel-Trénaunay syndrome.
MedLine Citation:
PMID:  11484474     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The Klippel-Trénaunay syndrome is a triad of congenital anomalies characterised by haemangiomas, varicosities, and unilateral bony and soft tissue hypertrophy. Hypertrophy usually affects one distal limb, but trunk or face may be affected. Cutaneous haemangiomas (nevus flammeus) of varying extent and irregular contour are often present in the hypertrophic regions. Varicosities may also be part of the vascular lesions of the syndrome. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Two cases of the Klippel-Trénaunay syndrome are presented here. Both of these show the typical hemifacial hypertrophy and premature eruption of teeth on the affected side. In the first case only the left mandibular region was affected. In contrast, in the second there was hypertrophy of the whole left side of the body including upper and lower jaws. This boy also suffers from congenital ideokinetic retardation, while the first was otherwise normal. Both cases differ from previously reported cases of the Klippel-Trénaunay syndrome in lacking any prominent facial nevus flammeus. In the first case there was also malformation of the crown of the first permanent molar on the affected side that has not been described previously.
Authors:
V Mueller-Lessmann; A Behrendt; W E Wetzel; K Petersen; D Anders
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  International journal of paediatric dentistry / the British Paedodontic Society [and] the International Association of Dentistry for Children     Volume:  11     ISSN:  0960-7439     ISO Abbreviation:  Int J Paediatr Dent     Publication Date:  2001 May 
Date Detail:
Created Date:  2001-08-03     Completed Date:  2001-09-20     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9107511     Medline TA:  Int J Paediatr Dent     Country:  England    
Other Details:
Languages:  eng     Pagination:  225-9     Citation Subset:  D    
Affiliation:
Department of Paediatric Dentistry, Dental Clinic, Justus-Liebig University, Schlangenzahl 14, D-35392 Giessen, Germany.
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MeSH Terms
Descriptor/Qualifier:
Child
Face / abnormalities*
Facial Asymmetry / pathology
Female
Hamartoma / pathology
Hemangioma / pathology
Humans
Klippel-Trenaunay-Weber Syndrome / pathology*
Male
Mandible / abnormalities
Maxilla / abnormalities
Mental Retardation / pathology
Molar / abnormalities
Mouth Abnormalities / pathology*
Skin Diseases / pathology
Skin Neoplasms / pathology
Tooth Crown / abnormalities
Tooth Eruption
Varicose Veins / pathology

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


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