Fibrous Dysplasia – DNB ENT Theory Question June 2015

June 2015 – 5 – a) Pathology & Types of Fibrous Dysplasia. b) Outline its management. (3+4+3)

Fibrous dysplasia:

• Genetically based tumor-like condition (not a true neoplasm).
• Normal medullary bone is replaced with fibro-osseous tissue.
• Commonly diagnosed within the first two decades of life.
• Self-limiting process and ceasing when affected bone reaches maximum growth and maturation.
• Reported cases of sarcomatous transformation.
• Juvenile variant:
  o Rapidly destructive, aggressive, destroys teeth, refractory to treatment.

Types:

1. Monostotic: 70-80%
    One bone involved.
    Most common form.
2. Polyostotic: 20-25 %
    More than one bone involved.
3. Disseminated (McCune-Albright Syndrome):
    Polyostotic fibrous dysplasia, precocious puberty,
   abnormal skin pigmented lesions (caft-au-lait).

Clinical picture:

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