Fibrous bone dysplasia

Fibrous bone dysplasia of the cheekbone (os zygomaticum)

Fibrous bone dysplasia (or Jaffé-Lichtensteinova disease) is a more common, non-hereditary disease characterized by the presence of expansive connective-bone tissue inside the diaphyses and metaphyses of growing bones (and possibly other symptoms).

Pathological anatomy[edit | edit source]

replacement of spongiosis of (mainly long) bones by high-cell fibrous tissue (dif. dg. fibrosarcoma) in young people
The connective tissue is sometimes arranged in a nodular manner, contains trabecular meshwork without osteoblastic borders
it is probably a bone tissue development disorder (stopped at the mesh bone stage, does not transform into lamellar bone)

Clinical picture[edit | edit source]

bones susceptible to pressure, tend to be deformed (shepherd's stick-shaped femur), tendency to pathological fractures
painful swelling, lameness, shortness of limbs, genu varum nebo valgum
occurrence in the form of monoostotic and polyostotic
McCune-Albright syndrome: association of the polyostotic form of fibrous dysplasia with foci of hyperpigmentation in the lumbar region, endocrine disorders (mainly pubertas praecox) and pathological changes in the hip joints (only in women)
familial fibrous dysplasia in the jaws leads to cherubism

X-ray image[edit | edit source]

irregular cystic confluent clearing within the bone ("milk glass")
thinning of the cortex

Therapy[edit | edit source]

excochleation, spongioplasty
corrective osteotomy, prolongation with an external fixator

Links[edit | edit source]

Used literature[edit | edit source]

DUNGL, P., et al. Ortopedie. 1. edition. Praha : Grada Publishing, 2005. ISBN 80-247-0550-8.