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Case Report
MELORHEOSTOSIS
Sclerosing mesodermal disease Derived from Greek:
Non-hereditary
melos limb
Benign
rhein to flow
Affects the skeleton and adjacent
osteon bone
soft tissue.
Mainly involves the lower
extremity, may also affect the
upper extremity, pelvic, costae,
and spine
Rarely involves the skull and
facial bones.
Characterized by a classic
radiographic feature of hyperostosis
periosteal on cortical of long bones
resembling dripping candle wax.
MELORHEOSTOSIS
Synonims = Candle wax diseases of the bones
Leris disease
Osteosis eburnisans monomelica.
A rare disease, first
DEMOGRAPHY OF
MELORHEOSTOSIS
About 400 cases have been
reported in the literature
CLINICAL MANIFESTATION
Chronic pain (neuralgia/arthralgia)
With periods of exacerbation & arrest
Intensity range from mild to severe
Limb stiffness
Restriction of joint movement
Deformity
CLINICAL MANIFESTATION
May involved:
segmental & unilateral hemimelic
1 bonemonostotic
1 limb monomelic
multiple bones polyostotic
Other disorder has been reported: Mesodermal
abnormalities such as hemangioma, nevi,
arteriovenosus malformation, glomus tumor.
CLINICAL MANIFESTATION
The osseus changes can cause functional morbidity.
Skin and subcutaneous tissue involvement:
Edema, hyperpigmentation, fibrosis, erythematous,
myositis, myosclerosis, muscular atrophy, joint
contracture that leads to deformity and limb
discrepancy which difficult to treat.
Treatment: Conservative and Operative,
multidisciplinary involving rheumatologist, medical
rehab and orthopaedic specialist.
CASE REPORT
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HISTORY TAKING
Chief complain: Pain on right knee, over 10 years, not
affected by the wheather, with periods of acutely
exacerbated by activity such as stair climbing and arrest.
He stated the pain felt like cramp and dull, initially. Then
turned into sharp and penetrating pain, radiated to the
ankle joint, upper thigh and the hip.
The pain gradually worsen since 2 years before,
accompanied by limb stiffness, leads to restriction of
movement on walking, hardly without any help.
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HISTORY TAKING
Initially, he went for traditional medicine. As he began hardly
walk, he went to an internist who stated that he suffered
from osteoporosis and was given Voltaren 2x50 mg. Up till
recently, he has been consuming the medication, not
routinely, and without control.
He reported no fever, cough or shortness of breath.
No nausea or vomiting. Still, he has had loss of appetite
since 2 years before, result in decrease of BW about 5 kg.
No complained of urinating and defecation.
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HISTORY TAKING
History of trauma 3 years before, which he fell from a
motorcycle. It caused a laceration and tender mass on his
right knee, which healed after he went for traditional
medication. There was also, lacerations on the right ankle.
No history of heart disease, HT, and diabetes.
No history of the same disorder in his family.
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PHYSICAL
EXAMINATION
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GALS EXAMINATION
Gait
Asymmetric
Waddling gait
Not able to turn quickly
Inspection
Mild scoliosis
from behind Paraspinal muscular normal and symmetric
Shoulder and gluteal muscular normal and symmetric
Iliacal height asymmetric (right higher than left)
No popliteal edema
Inspection
Cervical and lumbal lordosis normal
from lateral Mild thoracic kyphosis
Touch toes Spine and hip flexion not max (30o)
Palpation
Pain on lumbar area of supraspinatus, and right hip
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GALS EXAMINATION
Arms
Arms behind head Glenohumeral, sternoclavicular and
acromioclavicular joints movement normal
Arms straight
Elbow extension maximal
Hands in front
No edema/deformity on wrist & fingers
Fingers extension maximal
Turn hands over Supination/pronation normal, palmar normal (no
edema, muscular wasting & erythematous)
Make a fist
Fist strength normal
Fingers on thumb Fine precision normal/agile
Palpation
Pain on metacarpal (-)
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GALS EXAMINATION
Legs
Lying on a couch
Spine
Head on shoulders Lateral flexion of cervical normal
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LAB EXAMINATION
Leukocyte
6900
/mm3
Hb
Platelet
ESR
13.4
275000
7 / 16
g/dl
/mm3
/mm3
RBS
Ureum
Creatinine
SGOT/SGPT
104
17
0.7
16 / 21
mg/dl
mg/dl
mg/dl
mg/dl
Na/K/Cl
ALP
19
ECG
Sinus rhythm, heart rate 80
x/min
20
CLASSIC RADIOGRAPHIC
FEATURE
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Fig 3. Pelvic
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DIAGNOSI Melorheostosis
S
TREATMEN Meloxicam 15 mg/day
T
Calcium 2 tab/day
Informed & Educated
about progression, tx,
complication, &
prognosis
Referred to medical
rehab & orthopaedic
specialist.
26
DISCUSSION
Sclerotome - Murray & Mc Credie
(1979)
Melorheostosis lesion may correspond to a
sclerotome, which partially reflect the
monomelic tendency.
Hypothesi
s
27
Campbell et al
8 of 14 patients was
monomelic
Morris et al
81% of 131 patients
was monomelic
28
Classic radiography is
sufficient enough to
diagnose Melorheostosis
CT-scan and MRI are not
needed in the vast
majority of cases
Bone scintigraphy reveals
abnormal increased tracer
uptake in the bone and
soft tissue lesion
29
RADIOGRAPHY FEATURE
Pathognomonic:
Hyperostosis periosteal along the cortex of long
bones resembling melting wax flowing down the
side of a candle (the dripping candle wax sign)
Freyschmidt describe additional criterias:
1. Osteoma-like appearance with hyperostosis located either on the
outer or inner aspect of the affected bone
2. Osteopathia striata-like pattern, which showed long & dense
hyperostotic striations near the inner side of the cortex in 2 or more
bones
3. Myositis ossificans-like ossifications in the soft tissue in 2 or more
bones unilateral with/without intraosseous hyperostosis.
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RADIOGRAPHY FEATURE
1. Osteoma-like appearance
2. Osteopathia striata-like
pattern
3. Myositis ossificans-like
pattern
31
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33
Severe case
of
Melorheostos
is
Monomelic manifestation
34
LABORATORIUM
EXAMINATION
Lab result of this
Lab result of this
patient was normal.
35
DIFFERENTIAL
DIAGNOSIS
Osteochondroma
Parosteal osteosarcoma
Osteoid osteoma
Other sclerosing bone dysplasia:
- Osteopoikilosis
- Osteopathia striata
- Myositis ossificans
36
Osteoid osteoma
Osteochondroma
Parosteal osteosarcoma
37
Myositis ossificans
Osteopoikilosis
Osteopathia striata
38
TREATMENT
Each patient of Melorheostosis is
unique
39
TREATMENT OPTION
Konservati
ve
Pharmacolo
-
gy
Bisphosphonate
NSAID
Nifedipin
Nonpharmacology
- Physical
therapy
- Serial casting/splint
- Nerve block/symphatectomy
Operative
- Tendon shortening
- Fibrous & osseous
tissue excision
- Fasciotomy
- Capsulotomy
- Corrective osteotomy
- Bone debulking
- Amputation
40
41
Referred to rehab
specialist
42
PROGNOSIS
Despite of functional
morbidity, the prognosis
usually good.
43
SUMMARY
48 yo
Suffered from a rare disease, MELORHEOSTOSIS
Chronic pain over 10
years on the right
knee with periods of
exacerbation & arrest.
PE: waddling gait,
edema on right side of
lower limb, restriction
of joint movement,
with lower limb
discrepancy.
Symptomatic tx:
meloxicam 15 mg/day
& calcium 2 tab/day.
Informed & educated
about the progession,
comprehensive tx,
complication &
prognosis.
Referred to ortho &
med rehab.
Thank you!
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What does
LEMD3/MAN1 do?
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What does
LEMD3/MAN1 do?
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