Use of high-dose oral bisphosphonate therapy for symptomatic fibrous dysplasia of the skull

Case report

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Fibrous dysplasia of the bone in adults is a rare anomaly of skeletal development caused by a defect in differentiation of osteoblasts. This condition is associated with bone pain, bone deformity, and an increased incidence of fracture. Involvement of the skull is associated with headache along with dysmorphic features. Until recently, the principal treatment has been resection or fracture repair, although the latter is often palliative at best. However, new insight into the molecular mechanism of fibrous dysplasia has led to the use of bisphosphonates to treat this disease.

The authors examined the effects of high-dose oral alendronate (40 mg daily) for 6 months on 3 adult patients with intractable headache due to fibrous dysplasia of the skull. Each patient had disease processes not amenable to surgery. The patients underwent clinical follow-up at 1, 3, and 6 months. Their pain levels were documented at each visit by using a visual analog scale. All 3 patients demonstrated a significant decrease in pain levels and became independent of scheduled analgesics. Tumor bulk did not progress during this interval in any patient. Overall, alendronate was tolerated well, although in 1 patient it was discontinued early due to esophagitis. High-dose oral bisphosphonate therapy is an alternative therapeutic option for the palliative treatment of patients with fibrous dysplasia of the skull.

Article Information

Address correspondence to: Laurence Katznelson, M.D., 875 Blake Wilbur Drive, Stanford, California 94305-5826. email: LKatznelson@stanford.edu.

© AANS, except where prohibited by US copyright law.

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    Case 1. Noncontrast bone window head CT scans showing clear fibrous dysplasia involvement of the left orbit, anterior clinoid, and multiple sinuses.

References

  • 1

    Chapurlat R: Current pharmacological treatment for fibrous dysplasia and perspectives for the future. Joint Bone Spine 72:1961982005

    • Search Google Scholar
    • Export Citation
  • 2

    Chapurlat RD: Medical therapy in adults with fibrous dysplasia of bone. J Bone Miner Res 21:2 SupplP114P1192006

  • 3

    Chapurlat RDDelmas PDLiens DMeunier PJ: Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. J Bone Miner Res 12:174617521997

    • Search Google Scholar
    • Export Citation
  • 4

    Chapurlat RDHugueny PDelmas PDMeunier PJ: Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment. Bone 35:2352422004

    • Search Google Scholar
    • Export Citation
  • 5

    Colucci SMinielli VZambonin GCirulli NMori GSerra M: Alendronate reduces adhesion of human osteoclastlike cells to bone and bone protein-coated surfaces. Calcif Tissue Int 63:2302351998

    • Search Google Scholar
    • Export Citation
  • 6

    Dolgitser MStern LKatz LMDoyle JJOmar MSeton M: Bisphosphonate use in the treatment of Paget's disease of the bone: analysis of claims in a large database. Manag Care Interface 20:33402007

    • Search Google Scholar
    • Export Citation
  • 7

    Dumont ASBoulos PTJane JA JrEllegala DBNewman SAJane JA Sr: Cranioorbital fibrous dysplasia: with emphasis on visual impairment and current surgical management. Neurosurg Focus 10:5E62001

    • Search Google Scholar
    • Export Citation
  • 8

    Egner-Hobarth SWelkerling HWindhager R: [Bisphosphonates in the therapy of fibrous dysplasia: relevant data and practical aspects.]. Orthopade 36:1241302007. (Ger)

    • Search Google Scholar
    • Export Citation
  • 9

    Glorieux FHRauch F: Medical therapy of children with fibrous dysplasia. J Bone Miner Res 21:2 SupplP110P1132006

  • 10

    Khadilkar VVKhadilkar AVMaskati GB: Oral bisphosphonates in polyostotic fibrous dysplasia. Indian Pediatr 40:8948962003

  • 11

    Kitagawa YTamai KIto H: Oral alendronate treatment for polyostotic fibrous dysplasia: a case report. J Orthop Sci 9:5215252004

  • 12

    Kumar S: Bisphosphonate therapy in polyostotic fibrous dysplasia. Indian Pediatr 41:106910702004

  • 13

    Lane JMKhan SNO'Connor WJNydick MHommen JPSchneider R: Bisphosphonate therapy in fibrous dysplasia. Clin Orthop Relat Res 382:6122001

    • Search Google Scholar
    • Export Citation
  • 14

    Liens DDelmas PDMeunier PJ: Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet 343:9539541994

  • 15

    Mavrokokki TCheng AStein BGoss A: Nature and frequency of bisphosphonate-associated osteonecrosis of the jaws in Australia. J Oral Maxillofac Surg 65:4154232007

    • Search Google Scholar
    • Export Citation
  • 16

    Parisi MSOliveri BMautalen CA: Effect of intravenous pamidronate on bone markers and local bone mineral density in fibrous dysplasia. Bone 33:5825882003

    • Search Google Scholar
    • Export Citation
  • 17

    Reszka AARodan GA: Mechanism of action of bisphosphonates. Curr Osteoporos Rep 1:45522003

  • 18

    Riminucci MKuznetsov SACherman NCorsi ABianco PGehron Robey P: Osteoclastogenesis in fibrous dysplasia of bone: in situ and in vitro analysis of IL-6 expression. Bone 33:4344422003

    • Search Google Scholar
    • Export Citation
  • 19

    Riminucci MSaggio IRobey PGBianco P: Fibrous dysplasia as a stem cell disease. J Bone Miner Res 21:2 SupplP125P1312006

  • 20

    Schwartz DTAlpert M: The malignant transformation of fibrous dysplasia. Am J Med Sci 247:1201964

  • 21

    Stanton RP: Surgery for fibrous dysplasia. J Bone Miner Res 21:2 SupplP105P1092006

  • 22

    Weinstein LS: Gsα mutations in fibrous dysplasia and McCune-Albright syndrome. J Bone Miner Res 21:2 SupplP120P1242006

  • 23

    Yamada NTatsuno I: [McCune-Albright syndrome.]. Nippon Rinsho Suppl 2:Suppl1261302006. (Jpn)

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