RT Book, Section
A1 Wells, Robert G.
SR Print(0)
ID 1148954151
T1 Nonneoplastic Abnormalities of the Extremity Soft Tissues
T2 Diagnostic Imaging of Infants and Children
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071808392
LK accesspediatrics.mhmedical.com/content.aspx?aid=1148954151
RD 2024/04/19
AB Klippel-Trenaunay  syndrome  (Klippel-Trenaunay-Weber  syndrome;  angio-osteohypertrophy  syndrome)  is  a  developmental  vascular  abnormality  of  the  extremities.  Classically,  there  is  the  triad  of  cutaneous  capillary  malformations,  dilated  superficial  veins  or  venous  malformations,  and  overgrowth  of  the  limb  (local  gigantism).  Capillary-lymphaticovenous  malformations  are  present  in  the  deeper  soft  tissues  in  most  of  these  patients  as  well.  Typically,  the  abnormality  involves  a  single  lower  extremity  (95%);  arm  involvement  is  rare.  Klippel-Trenaunay  syndrome  apparently  is  due  to  a  disturbance  in  embryogenesis,  with  persistence  of  primitive  arteriovenous  communications  within  the  developing  limb  bud.  Abnormalities  of  superficial  veins  in  these  patients  range  from  ectasia  of  small  veins  to  large  venous  or  lymphaticovenous  malformations.  Potential  deep  venous  abnormalities  include  aplasia,  hypoplasia,  aneurysmal  dilation,  duplications,  and  valvular  incompetence.1–3