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Melorheostosis
1.
2. 1
• Also known as Leri disease
2
• Is an uncommon mesenchymal dysplasia
3
• Regions of sclerosing bone with a
characteristic dripping wax appearance or
flowing candle wax appearance
3. changes in:
early childhood
age
Presentation:
often later
condition often
remains occult
until late
adolescence or
early adulthood
No recognised
familial
predisposition
In half of cases
:diagnosis
before the age
of 20
4. Clinical presentation
• The condition, especially in childhood, is usually
asymptomatic, with the condition diagnosed as an
incidental finding on radiographs obtained for
another purpose
• When melorheostosis does manifest clinically,
the most common presentation is of limb
stiffness (flexion contractures) or pain.
•This is more common in adults.
5. •One of a group of sclerosing bone disorders
•Rare
1
•Cause is unknown
•Produces thickening of the endosteum and periosteum
2
•Peak age of presentation is 5-20 years
•May be monostotic or polyostotic
•May involve one entire limb
•Usually does not involve multiple limbs
3
•Twice as common in lower extremities than elsewhere
6. About 50% affected
develop symptoms by
age 20
Pain
Adults
present with
Joint stiffness
Children may
present with
Joint contractures
Leg length
discrepancies
Deformity that may
progress over time
7. sclerodermic skin changes : thickening & fibrosis of overlying skin
vascular tumours and malformations
muscle atrophy
It has a predilection for long bones of the limbs ,it can be seen
almost anywhere
Hands and feet are not infrequently involved ,and involvement of the
axial skeleton is rare
9. Sclerotic lesions of cortical bones, usually in the diaphysis,
that resemble “candle-wax-dripping”
Cortical hyperostosis with an undulating appearance usually
affecting one side of a bone
Soft tissue lesions that may calcify (Adjacent to involved bone)
May grow to compress nerves
Usually low signal on MRI (Enhance with Gadolinium)
Bone scan is markedly positive
19. The disease is of variable severity
• chronic progressive course in adults
faster course in children
• Resulting in substantial disability from
contractures or deformity.