title: How do the type and location of a vascular malformation influence growth in Klippel-Trénaunay syndrome?
abstract: BACKGROUND
  Although Klippel-Trénaunay syndrome is a mixed vascular malformation characterized by abnormal growth in the extremities, no uniform diagnostic criteria have been established because of the variety in its manifestation. Consequently, no anatomical analysis based on a comparison study has been reported. In this study, the authors determine the frequency of various vascular malformations and abnormal growth and assess any statistical relationship between vascular malformation type/location and abnormal growth in terms of length and girth.
  
  
  METHODS
  Thirty-five patients with Klippel-Trénaunay syndrome satisfying the criteria proposed by Oduber et al. in 2008 were enrolled. The type and location of the vascular malformation and abnormal circumferential growth were assessed by magnetic resonance imaging and ultrasonography. Bone girth was assessed by axial magnetic resonance imaging/computed tomography. Plain radiographs of the long bones were used to measure growth in length.
  
  
  RESULTS
  The spectrum of vascular types was similar to that in previous reports. There was no significant association between leg length and vascular malformation type or location. Leg bone circumferential hypoplasia was observed in 50 percent of cases and was significantly related to the presence of intramuscular lesions. A single venous malformation in the subcutaneous tissue was significantly associated with the presence of subcutaneous hypertrophy. Patients with intramuscular lymphatic malformations had a significantly higher frequency of muscle hypoplasia.
  
  
  CONCLUSIONS
  The type and location of certain vascular malformations were significantly associated with abnormal subcutaneous tissue, muscle, and bone growth.
authors.affiliation: Sapporo, Japan From the Departments of Plastic and Reconstructive Surgery of Hokkaido University Graduate School of Medicine and KKR Sapporo Medical Center, Tonan Hospital.
authors.forename: Emi
authors.initials: E
authors.lastname: Funayama
authors.forename: Satoru
authors.initials: S
authors.lastname: Sasaki
authors.forename: Akihiko
authors.initials: A
authors.lastname: Oyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Furukawa
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Hayashi
authors.forename: Yuhei
authors.initials: Y
authors.lastname: Yamamoto
mesh_terms: D000293:Adolescent; D000328:Adult; D001808:Blood Vessels / Q000002:abnormalities* / Q000473:pathology; D001846:Bone Development / Q000502:physiology*; D001842:Bone and Bones / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006984:Hypertrophy; D007223:Infant; D007715:Klippel-Trenaunay-Weber Syndrome / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D007866:Leg / Q000033:anatomy & histology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D040521:Subcutaneous Tissue / Q000098:blood supply / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D054079:Vascular Malformations / Q000473:pathology; D014680:Veins / Q000002:abnormalities