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