title: Inverse Klippel-Trenaunay syndrome: review of cases showing deficient growth.
abstract: BACKGROUND
  Klippel-Trenaunay syndrome is defined by a coexistence of nevus flammeus and overgrowth of one or more limbs. Remarkably, however, deficient growth of an affected limb may likewise be noted.
  
  
  METHODS
  We collected from the literature a number of cases of Klippel-Trenaunay syndrome associated with deficient growth such as shortening or hypoplastic muscle mass of the affected extremity.
  
  
  CONCLUSIONS
  The cause of the unusual deficient growth is unknown. Some patients may be compound heterozygotes carrying a 'plus' and a 'minus' allele at the responsible gene locus, and postzygotic recombination would give rise to two different cell clones homozygous for either allele.
  
  
  CONCLUSIONS
  In order to give a name to such paradoxical cases, we propose the term 'inverse Klippel-Trenaunay syndrome'.
authors.affiliation: Department of Dermatology, Philipp University of Marburg, Marburg, Germany.
authors.forename: Retno
authors.initials: R
authors.lastname: Danarti
authors.forename: Arne
authors.initials: A
authors.lastname: König
authors.forename: Mario
authors.initials: M
authors.lastname: Bittar
authors.forename: Rudolf
authors.initials: R
authors.lastname: Happle
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006130:Growth Disorders / Q000150:complications* / Q000235:genetics; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000150:complications / Q000235:genetics; D013577:Syndrome; D009626:Terminology as Topic