1. Melorheostosis is a rare mesenchymal dysplasia that produces thickened bone with a characteristic dripping wax appearance, most commonly affecting the limbs. 2. It typically presents in childhood and adolescence between ages 5-20 years as limb stiffness or pain. 3. While the condition is usually asymptomatic, when symptoms do occur they include joint stiffness, contractures, pain and limb length discrepancies.

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

8. Resembling osteoma(s)
Candle-wax appearance (classic)
Resembling myositis ossificans
Resembling osteopathia striata
Mixed

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

13. Case 3 : monostotic
melorheostosis

14. Case 5 :
involving radius

15. Case 6 :
involving ribs

16. Increase in radiotracer uptake is usually
present on late phase bone scans

17. • Osteopathia striata
1
• Longitudinal dense striations
• Osteopoikilosis
2
• Punctate, rounded bone islands
surrounding joints
• Osteosarcoma
3
• Bone destruction

18. Associated soft tissue lesions and cutaneous
lesions
Vascular malformations, Neurofibromatosis,
Tuberous sclerosis, Hemangioma
Muscle contractures
Scoliosis

19. The disease is of variable severity
• chronic progressive course in adults
faster course in children
• Resulting in substantial disability from
contractures or deformity.

20. Conservative
•Conservative
management is
often unrewarding
surgical
intervention
•including tendon
release, osteotomies
and even amputation