title: A proposal for classification of entities combining vascular malformations and deregulated growth.
abstract: BACKGROUND
  Agreement on terminology and nomenclature is fundamental and essential for effective exchange of information between clinicians and researchers. An adequate terminology to describe all patients showing vascular malformations combined with deregulated growth is at present not available.
  
  
  OBJECTIVE
  To propose a classification of patients with vascular malformations, not restricted to the face, and growth disturbances based on simple, clinically visible characteristics, on which clinicians and researchers can comment and which should eventually lead to an internationally accepted classification.
  
  
  METHODS
  Rooted in our joint experience we established a classification of vascular malformation not limited to the face, with growth disturbances. It is based on the nature and localization of the vascular malformations; the nature, localization and timing of growth disturbances; the nature of co-localization of the vascular malformations and growth disturbances; the presence or absence of other features. Subsequently a mixed (experienced and non-experienced) group of observers evaluated 146 patients (106 from the Netherlands; 40 from the UK) with vascular malformations and disturbed growth, using the classification. Inter-observer variability was assessed by estimating the Intra-Class Correlation (ICC) coefficient and its 95% confidence interval.
  
  
  RESULTS
  We defined 6 subgroups within the group of entities with vascular malformation-deregulated growth. Scoring the patients using the proposed classification yielded a high inter-observer reproducibility (ICC varying between 0.747 and 0.895 for all levels of flow).
  
  
  CONCLUSIONS
  The presently proposed classification was found to be reliable and easy to use for patients with vascular malformations with growth disturbances. We invite both clinicians and researchers to comment on the classification, in order to improve it further. This way we may obtain our final aim of an internationally accepted classification of patients, which should facilitate both clinical treatment and care of, as well as research into the molecular background of entities combining vascular malformation and deregulated growth.
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Charlène E U
authors.initials: CE
authors.lastname: Oduber
authors.forename: Chantal M A M
authors.initials: CM
authors.lastname: van der Horst
authors.forename: J Henk
authors.initials: JH
authors.lastname: Sillevis Smitt
authors.forename: Mark J C
authors.initials: MJ
authors.lastname: Smeulders
authors.forename: Vibhu
authors.initials: V
authors.lastname: Mendiratta
authors.forename: John I
authors.initials: JI
authors.lastname: Harper
authors.forename: Maurice A M
authors.initials: MA
authors.lastname: van Steensel
authors.forename: Raoul C M
authors.initials: RC
authors.lastname: Hennekam
mesh_terms: D000328:Adult; D000368:Aged; D001132:Arm / Q000098:blood supply; D002196:Capillaries / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005145:Face / Q000098:blood supply; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007866:Leg / Q000098:blood supply; D008208:Lymphatic System / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D054079:Vascular Malformations / Q000145:classification*; D014680:Veins / Q000473:pathology*; D055815:Young Adult