Pamidronate treatment of polyostotic fibrous dysplasia: failure to prevent expansion of dysplastic lesions during childhood.

Abstract

AIMS:

To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS).

METHODS:

Radiological evidence of fibrous dysplasia progress was reviewed for three children with MAS who were treated with pamidronate from age 2.5-5 years, for 8-10.5 years.

RESULTS:

Despite minimal pain and a low fracture rate in long bones, except where gross deformity exists, all dysplastic lesions present in long bones continued to undergo uncontrolled expansion. In contrast, there were no major new changes in facial configuration, no clinically obvious expansion of sphenoid wing lesions and no encroachment on optic foramina or visual field restriction in any patient.

CONCLUSIONS:

Despite previous reports of limitation or reduction in size of fibrous dysplasia lesions in adults and children, it is our experience that bisphosphonate treatment of polyostotic fibrous dysplasia in children with MAS does not arrest the expanding nature of these lesions.

• Authors: Brendan Chan, Margaret Zacharin
• Journal: Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) Vol. 19 Issue 1 Pg. 75-80 (Jan 2006) ISSN: 0334-018X [Print] Germany
• PMID: 16509531 (Publication Type: Case Reports, Journal Article)

Chemical References

• Bone Density Conservation Agents
• Diphosphonates
• Pamidronate

Topics

• Adolescent
• Bone Density Conservation Agents (therapeutic use)
• Bone and Bones (diagnostic imaging, drug effects)
• Child
• Child, Preschool
• Diphosphonates (therapeutic use)
• Disease Progression
• Fibrous Dysplasia, Polyostotic (diagnostic imaging, drug therapy, prevention & control)
• Follow-Up Studies
• Humans
• Male
• Pamidronate
• Radiography
• Treatment Failure