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Klippel-Trenaunay-Weber Syndrome 759.89

 

Klippel-Trenaunay-Weber ICD-9

  • 759.89 Congenital malformation syndromes affecting multiple systems

Klippel-Trenaunay-Weber Natural History, Etiology

  • venous malformations in association with focal overgrowth’s syndrome
  • Autosomal Dominant
  • short-trunked, disproportionate dwarfism
  • defect in gene for type II collagen

Klippel-Trenaunay-Weber Clincal Evaluation

  • joint stiffness/contractures
  • scoliosis
  • kyphosis
  • dumbbell-shaped femora
  • hypoplastic pelvis/spine
  • Respiratory problems
  • cleft palate
  • otitis meida/hearing loss are common

Klippel-Trenaunay-Weber Xray

  • Osteopenia
  • dumbbell shaped bones

Klippel-Trenaunay-Weber Treatment

  • opthomology consult for retinal detachment / myopia
  • Physical Therapy for joint contractures
  • Reconstruction for early hip DJD