title: Poor results of drilling in early stages of juxta-articular osteonecrosis in 12 joints affected by Gaucher disease.
abstract: OBJECTIVE
  Gaucher disease is heterogeneous. One of the most devastating complications is bone involvement, ranging from mild osteopenia to osteonecrosis, but no markers have been discovered to predict onset and/or progression. We describe our experience in a large referral center using drilling for juxta-articular osteonecrosis in young patients with Gaucher disease.
  
  
  METHODS
  We retrospectively reviewed medical data from all patients who were recommended to undergo drilling for osteonecrosis of juxta-articular bone of the femoral head, the humeral head, or upper tibia for acute osteonecrosis at a pre-collapse stage.
  
  
  RESULTS
  11 patients (mean age 34 years) underwent drilling of 12 joints with juxta-articular osteonecrosis; 3 (mean age 51 years) refused intervention. 9 joints that were drilled showed advancing joint degeneration within 0.5 to 4 years. 3 joints have undergone replacement. Of the 3 joints that did not undergo drilling, 2 have undergone replacement and 1 has collapsed with osteoarthritis.
  
  
  CONCLUSIONS
  We found equally poor outcome with and without drilling. Effective intervention can only be achieved by improving our understanding of bone physiology and pathophysiology in Gaucher disease.
authors.affiliation: Department of Orthopaedic Surgery, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Ehud
authors.initials: E
authors.lastname: Lebel
authors.forename: Mici
authors.initials: M
authors.lastname: Phillips
authors.forename: Deborah
authors.initials: D
authors.lastname: Elstein
authors.forename: Ari
authors.initials: A
authors.lastname: Zimran
authors.forename: Menachem
authors.initials: M
authors.lastname: Itzchaki
mesh_terms: D000328:Adult; D019643:Arthroplasty, Replacement; D005260:Female; D005271:Femur Head Necrosis / Q000601:surgery; D005500:Follow-Up Studies; D005776:Gaucher Disease / Q000150:complications / Q000601:surgery*; D006801:Humans; D006811:Humerus / Q000473:pathology / Q000601:surgery; D007596:Joints / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D019637:Orthopedic Procedures / Q000379:methods; D010020:Osteonecrosis / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome