Overgrowth management in Klippel-Trenaunay-Weber and Proteus syndromes.
Twenty-eight patients with limb overgrowth and the diagnosis of Klippel-Trenaunay-Weber or Proteus syndromes were evaluated retrospectively. These disorders are part of the phakomatosis spectrum of syndromes. The orthopedic problems consisted of asymmetric limb overgrowth, localized gigantism, angular deformities, scoliosis, vascular malformations, and skin anomalies. Systemic abnormalities are common and deserve full evaluation before treatment. Surgical treatment consisted of epiphysiodesis, osteotomies, debulking procedures, and amputation. Mixed results were obtained with surgery, and conservative or supportive treatment should be the primary mode of orthopedic care.
Guidera
K J
KJ
Shriners Hospital for Crippled Children, Tampa Unit, FL 33612-9499.
Brinker
M R
MR
Kousseff
B G
BG
Helal
A A
AA
Pugh
L I
LI
Ganey
T M
TM
Ogden
J A
JA
D000293:Adolescent; D000671:Amputation, Surgical; D001842:Bone and Bones / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003238:Connective Tissue / Q000473:pathology; D005260:Female; D005532:Foot Deformities, Congenital / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006228:Hand Deformities, Congenital / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008297:Male; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D016715:Proteus Syndrome / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D014057:Tomography, X-Ray Computed