A 47-year-old male presented with a history of left foot pain, discomfort, difficulty standing and walking, and a sensation of "walking on marbles" for several months; he also was unable to fit his foot into his shoe. The patient's past medical history was significant for left foot sesamoiditis and remote surgical procedures on the left foot.
A 47-year-old male presented with a history of left foot pain,
discomfort, difficulty standing and walking, and a sensation of
"walking on marbles" for several months; he also was unable to fit
his foot into his shoe. The patient's past medical history was
significant for left foot sesamoiditis and remote surgical
procedures on the left foot. A plain radiograph of the left foot
showed mixed sclerosing dystrophy in addition to a
"candle-wax-like" appearance and thickening of the cortices of the
calcaneum, third through fifth metatarsals, and proximal phalanges.
An MRI scan showed signal alteration and hypointensity on both T1-
and T2-weighted sequences. A three-phase bone scan with Tc-99m-MDP
scintigraphy demonstrated moderately intense uptake of the
radiopharmaceutical localized to the left lateral foot, which
corresponded to the sclerosing lesion seen on the plain radiographs
Melorheostosis of the left foot.
The plain radiographs showed flowing hyperostosis and
candle-wax-like thickening of the cortices involving the third
through fifth metatarsophalangeal joints, proximal phalanges, and
calcaneus of the left
foot. Three-phase bone scintigraphy demonstrated increased
vascular flow and blood pool activity, with associated significant
osseous uptake on delayed static images involving the lateral half
of the left foot extending from the proximal metatarsophalangeal
joints to the calcaneus. MRI showed hyperostosis with uniform
hypointensity on all imaging sequences involving the calcaneus and
third, fourth, and fifth digits. The pathology report confirmed the
clinical and imaging diagnosis and revealed extensive bone
remodeling showing both osteoclastic and osteoblastic activity,
with associated ossification of fibrous tissue filling the
trabecular space consistent with melorheostosis.
Melorheostosis is a rare form of mixed sclerosing bone
dysplasia. The name is derived from the Greek term melos (limb) and
rhein (to flow). In children, it is characterized by dense linear
streaks, mostly in the inner cortex of the affected bones. In
adults, irregular ossification is seen along the outer cortex,
mimicking candle drippings. Patients with melorheostosis may
develop secondary symptoms related to limitation of motion, joint
fusion, or periosteal irritation.1,2 The etiology is obscure but
Murry and McCreide3 suggested that an infection associated with
nerve roots may be responsible for this rare condition. They found
that the disorder most often involves one or more segmental
sclerotomes, areas of a bone innervated from an individual spinal
sensory nerve. This may in part explain the monomelic and linear
track involvement and distribution of melorheostoses.
A well known feature of hyperostotic bone remodeling is its
distinctive appearance, resembling wax melting and dripping down
one side of a candle. Several cases of this sign have been reported
from antiquity; the skeleton of a 50-year-old Alaskan Eskimo from
the fifth century A.D. and a 25- to 30-year-old female dating back
to 4000 to 5000 B.C. from Northern Chile both showed hyperostotic
lesions resembling candle wax.4 Despite this characteristic
feature, atypical presentations, particularly forme fruste lesions,
raise uncertainties about the diagnosis. Forme fruste lesions are
considered the mildest manifestation of this condition, which may
only focally involve the bone (monostotic) and, as such, is
difficult to differentiate from a focus of myositis ossifications
or periosteal osteosarcoma.5
Melorheostosis can be found in all age groups but is most
frequently diagnosed in young adults. Contracture of joints,
fibrosis of soft tissue, and changes in the skin may be present at
birth,6 but in these cases definitive diagnosis generally is
delayed until osseous abnormalities appear. There is no sexual
predilection in the disorder. In 70 to 80% of cases, melorheostosis
is monomelic and affects one or more contiguous bones.7 It is more
common in lower extremities and is usually appendicular. Other
possible sites include the skull, spine, ribs, and pelvic bones.8,9
The disorder usually is asymptomatic in children; in adults, it
presents with osseous complaints, usually beginning in the second
and third decades of life. Occasionally, it may present with one or
more palpable masses. Most of the clinical signs of the osseous
dysplasia are associated with fibrosis, which is usually the cause
for joint stiffness, angular joint deviations, contractures (mostly
flexion), and other orthopedic problems.8,10 Skin pigmentation,
scleroderma, and tuberous sclerosis-like thickening have been
described.11,12 In rare instances, melorheostosis has been reported
in association with tumors such as spinal column lipomatous
osteosarcoma, and soft-tissue lesions such as desmoid tumor.
Routine laboratory studies usually are unremarkable.
Histologically, sclerosis, soft-tissue fibrosis, and
periarticular calcification may be seen, and though these may lead
to bony ankylosis of the involved joint, none of these signs are
pathognomonic of melorheostosis. Differential diagnosis for this
condition includes osteomyelitis, reflex sympathetic dystrophy,
neurofibromatosis, soft-tissue sarcoma, and metastatic lesions.
This patient's plain radiographs are characteristic (figure 1),
showing wavy cortical hyperostosis which extends along the length
of the bone, resembling flowing candle wax. Magnetic resonance
(figure 2) features of melorheostosis include hyperostosis,
which appears as uniform hypointensity on all imaging sequences.
The Tc-99m-MDP three-phase bone scintigraphy image (figure 3)
demonstrates moderate to severe increase in tracer in the early and
late images, including soft-tissue and blood pool stages of
The course of this condition is unrelenting; however, it does
slow considerably in adulthood. Treatment usually is symptomatic,
though surgical measures such as osteotomies, excision,
capsulotomies, or tendon lengthening often are pursued.
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3. Murry RD, McCreide J: Melorheostosis and sclerotomes.
Skeletal Radiology 4:57-66, 1979.
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forme fruste of melorheostosis. Clin Nucl Med 19:512-515, 1994.
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Br 61:415, 1979.
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association with osteodystrophies-
a spectrum. Skeletal Radiol 10:95-101, 1983.
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report of the clinical roentgenographic and pathologic findings in
fourteen cases. J Bone Joint Surg Am 26:1281, 1968.
9. Ippolito V, Mirra JM, Motta C, et al: Case report 771:
Melorheostosis in association with desmoid tumor. Skeletal Radiol
10. Dissing I, Zafirovski G: Para-articular ossification
associated with melorheostosis Leri. Acta Orthp Scand 50:717-719,
11. Morris JM, Samilson RL, Corley SC: Melorheostosis. J Bone
Joint Surg Am 45:1191, 1963.
12. Perlman MD: Melorheostosis: A case report and a literature
review. J Foot Surg 29:353,
Prepared by Amir Salmanzadeh, MD, William Beaumont Hospital,
Royal Oak, MI, Stephen J. Pomeranz, MD, Parshan S. Ramsingh, MD,
and Jyotsna Rao, MD, Department of Nuclear Medicine, Metabolic
Imaging, and Radiology, The Christ
Hospital, Cincinnati, OH.