What are the possible complications of polyostotic fibrous dysplasia (PFD) in McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Answer

Lesions in FD range from relatively benign and asymptomatic to serious and debilitating, depending on the location. Lesions in weight-bearing bones can cause pathologic fractures. Depending on the specific bone involved and the specific location, potential complications of fractures include secondary osteomyelitis, compressive neuropathy, Volkmann contractures, sympathetic algodystrophy (reflex sympathetic dystrophy), myositis, ligamentous ossifications, and pseudoarthrosis.

The most dreaded complication of PFD is osteosarcoma, which most often occurs in the setting of irradiation of PFD-affected bones. [34] It is very uncommon; the overall incidence of sarcomatous degeneration in the setting of PFD is less than 1%. Most frequently, it involves the bones of the face and femur.

FD in the skull can be quite disfiguring and may be associated with blindness as a consequence of optic nerve compression. [35] Deafness also can occur and is associated with vestibulocochlear nerve compression. Other potential complications can result from compressive neuropathies of the cranial nerves located at the base of the skull. Rarely, compression fractures in the spine with impingement on spinal nerves have been reported.


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