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Kniest’s Syndrome 259.4

 

Kniest's Syndrome ICD-9

  • 259.4 Dwarfism, not elsewhere classified

 Kniest's Syndrome Natural History, Etiology

  • Autosommal Dominant
  • short-trunked dispropoinate dwarfism
  • defect in type II collagen

Kniest’s Clincal Evaluation

  • joint stiffness/contractures
  • scoliosis
  • kyphosis
  • dumbbell shaped femora
  • hypoplastic pelvis/spine
  • respiratory problems
  • cleft palate
  • otitis media, hearing loss

Kniest’s Treatment

  • optho consult(retinal detachment, myopia)
  • Physical Therapy
  • recon for DJD

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