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PubMed1994

Radionuclide imaging in forme fruste of melorheostosis.

Scintigraphy was instrumental in two histologically proven cases of forme fruste melorheostosis. Radionuclide bone scans demonstrated a moderately increased uptake of radiopharmaceutical localized to the "flowing" cortical hyperostosis of melorheostosis observed radiographically. The medullary portion of the affected bones showed no increase in tracer activity. In one case, imaging with Tl-201 chloride demonstrated increased focal activity of the lesion. These scintigraphic findings can help distinguish the mildest manifestation (forme fruste) of melorheostosis from the well-ossified lesions of myositis ossificans and parosteal or periosteal osteosarcoma. The findings of MRI are also described in one patient.

Adult↗

PubMed1998

Atypical decubital fibroplasia in a young patient with melorheostosis.

A case of atypical decubital fibroplasia of the right forearm arising in a 25-year-old male with melorheostosis is presented. The diagnosis of melorheostosis involving the right-sided bones was made by radiographical studies, and the patient has been obliged to use crutches due to the contracture and limited range of motion of the right leg. Two painless masses occurred in the subcutis of the posterior aspect of the right forearm over the excrescences of the underlying ulna due to melorheostotic deformity. Grossly, ill-defined firm masses, which measured 3 x 6 x 1.5 cm and 4 x 5 x 1 cm, respectively, were white and intermingled with yellow fatty tissue. Histologically, the lesions consisted of a proliferation of plump fibroblastic cells with abundant collagenous stroma. Vascular proliferation and occasional eosinophilic degeneration of the collagen fibers were also seen. The gross and histological features were those of atypical decubital fibroplasia (ischemic fasciitis). Immunohistochemically, the plump fibroblastic cells were positive for vimentin, but negative for desmin, muscle specific actin, and alpha-smooth muscle actin. Chondroid metaplasia was focally noted and round-shaped cells within this area were positive for S-100 protein. This lesion seemed to be a fibroblastic response against the long-standing, intermittent ischemia of the subcutaneous tissue between the bony excrescences due to melorheostosis and the weight-bearing forces of the crutch.

Adult↗

PubMed1978

99mTc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata, and melorheostosis.

Technetium-99m pyrophosphate bone images were contrasted with radiographic findings in two patients with osteopoikilosis, one with osteopathia striata, and one with melorheostosis. Despite the striking radiographic findings, scans were normal in osteopoikilosis and osteopathia striata. In melorheostosis, focal increased radiopharmaceutical accumulation appeared in each radiographically abnormal area. Technetium-99m pyrophosphate bone images should prove helpful when superimposed osseous disease must be differentiated from osteopoikilosis, osteopathia striata, or melorheostosis.

Adult↗

PubMed1999

Bone dysplasia series. Melorheostosis: review and update.

Melorheostosis is a rare nongenetic developmental anomaly first described in 1922 by Léri and Joanny. Its etiology is unknown. Patients present at any age, and both sexes are affected equally. Onset is usually insidious, with deformity of the extremity, pain, limb stiffness and limitation of motion in the joints first manifesting in late childhood or early adolescence and progressing into adult life. The characteristic radiographic appearance consists of irregular hyperostotic changes of the cortex, generally on one side of the bone, resembling melted wax dripping down one side of a candle. This appearance gave the anomaly its name, which is taken from the Greek words for member (melos) and flow (rhein). There is usually a distinct demarcation between the affected and normal bone. Dense, sclerotic linear areas are seen mainly in the cortex but also extending into the cancellous bone. Melorheostosis affects mainly the long bones of the upper and lower limbs, but also the short bones of the hand and foot and, rarely, the axial skeleton. It may co-exist with osteopoikilosis and osteopathia striata as well as with tumours or malformations of blood vessels or lymphatics. Soft-tissue ossifications at the site of the joint are common. Bone scintigraphy is positive and shows moderately increased uptake of tracer. Computed tomography and magnetic resonance imaging can further characterize the lesion, but rarely contribute to the diagnosis. The forme fruste of melorheostosis may mimic other conditions such as myositis ossificans, osteoma and parosteal osteosarcoma. Treatment of this chronic and sometimes debilitating condition consists of surgical soft-tissue procedures and even, in very severe cases, amputation.

Adolescent↗

PubMed2004

Melorheostosis of Leri: report of a case in a young African.

UNLABELLED: Melorheostosis of Leri is a non-familial condition of hyperostosis of the cortical bone that usually presents unilaterally in long bones of the upper and lower limbs, but may also present in vertebra, ribs, skull and jaw. The incidence of this disease is quite rare, only about 300 cases have been reported worldwide. We present a case, which may be the first documented case in sub-Saharan Africa. CLINICAL PRESENTATION: S.K. is a 14 year old male student who presented to the hospital with an 18 month history of persistent pain in the joints of the right upper limb and a limb length discrepancy since birth which has worsened with growth. Examination revealed generalised hypoplasia of the right upper limb with shortening of the limb and atrophy of the muscles, also hypoplasia and contracture of the thumb was observed. The radiographs of the limb showed multiple areas of dense hyperostosis and scleroderma, which showed a linear distribution along the radial half of the bones. CONCLUSION: In children presentation of melorheostosis, is more likely be as limb length discrepancy, deformity or joint contractures which may be seen before radiographic evidence of any bony changes. Improvement in imaging techniques will therefore result in early diagnosis and greater success with conservative management. Also the increased frequency of tumours necessitates long-term follow up. KEYWORDS: melorheostosis, scleroderma.

Adolescent↗

PubMed1986

Synchronous left subclavian and axillary artery aneurysms associated with melorheostosis.

Melorheostosis is a benign, rare congenital disorder of hyperostosis of one or more bones. A case of melorheostosis associated with synchronous left subclavian and axillary artery aneurysms necessitated resection of the aneurysms and replacement with a Gore-Tex interposition graft (W. L. Gore & Associates, Inc., Elkton, Md.) and a reversed saphenous vein graft, respectively. Numerous soft-tissue and vascular anomalies have been noted in patients with melorheostosis, although arterial aneurysms have not been reported previously.

Aneurysm↗

PubMed1995

[A case of melorheostosis with linear sclerodermatous skin changes].

A 69-years old Japanese woman complained of pain in the left elbow joint and thickened skin over the left upper limb. The pain had been present for 20 years, and the thickened area of the skin gradually enlarged during this period. Her left elbow joint showed some limitation of motion. There was no record of any similar condition in her family history. Radiographs of the left limb showed cortical hyperostosis extending from the middle of the left humerus to the distal end of the radius. Radiographs of the other limbs were normal. A technetium 99m-methylene diphosphonate bone scintigraphy revealed increased uptake in the areas of radiographic hyperostosis. The diagnosis of melorheostosis was made. Skin biopsy of thickened area was performed. The epidermis was normal, and proliferation of normal-appearing collagen fibers into the subcutaneous fat was noted. No inflammatory changes were found. The cause of sclerodermatous skin changes was thought to be not by linear scleroderma but by melorheostosis. In cases of linear sclerodermatous changes, melorheostosis as its origin should be considered.

Aged↗

PubMed1993

[Association of monomelic melorheostosis and synovial chondromatosis of the knee].

Melorheostosis is a rare affection, of obscure etiopathogenesis, corresponding to osteosclerosis with running hyperostosis. We report the observation of melorheostosis of a lower limb occurring to an 18-year old patient who consulted for tumefaction of the lower limb with an aching and stiffened knee. The radiography allowed the diagnosis by showing an aspect characterized by a running like of a candle accompanied by osteochondromatosis of the knee and the calcification of soft tissues. The synovectomy of the knee brought about indolence and the lesions seem to stabilise after a 3 years follow-up. The synovial osteochondromatosis seems to be an integral part of the disease. The characteristics of melorheostosis are studied according to the data of the literature.

Adolescent↗

PubMed2003

Melorheostosis in a family with autosomal dominant osteopoikilosis: report of a third family.

We describe a three-generation family with clinical and radiological findings of osteopoikilosis in five and melorheostosis in one individual. The co-occurrence of both rare bone disorders suggests that both conditions might be related as suggested previously by Butkus et al. [1997: Am J Med Genet 72:43-46] and Nevin et al. [1999: Am J Med Genet 82:409-414]. The findings in this family strengthen the hypothesis that osteopoikilosis is an autosomal dominant condition and that an early postzygotic second hit mutation in the second allele results in melorheostosis.

Child↗

PubMed2005

Coexistence of melorheostosis and DISH in a female skeleton from Magna Graecia (Sixth Century BC).

This paper reports on a case of massive hyperostotic alterations observed in the skeleton of an adult woman from the necropolis of Montescaglioso Belvedere (Basilicata, Southern Italy) attributed to the Enotrian culture and dated to the 6th century BC. Hyperostotic changes involve joints, the vertebral column, and the lower limbs. In particular, the large flowing ossification in both the thoracic (T6-T10) and lumbar (L2-L5) tracts, the sacralization of L5, accompanied by sacroiliac fusion on the left side, and the proliferative bone production on both the metaphyseal portion of the left tibia and the left third metatarsal are described. The vertebral antero-lateral ossification and the sacroiliac fusion support a diagnosis of diffuse idiopathic skeletal hyperostosis (DISH), while the flowing bone formation on the lower limbs supports a diagnosis of melorheostosis. The pathological conditions described here were already described in the literature, but this is the first reported case of the coexistence of DISH and the very rare melorheostosis. Furthermore, the skeleton from Montescaglioso Belvedere represents the first case of this disease described for ancient Europe, therefore adding a valuable contribution to the reconstruction of the antiquity and distribution of skeletal dysplasias.

Female↗

PubMed1979

Melorheostosis and the sclerotomes: a radiological correlation.

Melorheostosis is characterised radiologically by hyperostotic linear densities in bone. These densities have a peculiarly segmental distribution which does not correspond with the anatomical course of blood vessels or mixed nerve roots of the limbs. So far this disbritubion has lacked any valid explanation, although it has been suggested to be a developmental error as a result of an embryonic metameric disturbance. Inman and Saunders in 1944 described a sensory nerve supply to skeletal structures with 'sclerotomes' representing the zones of the skeleton supplied by individual spinal sensory nerves. Radiographs of 30 cases from the Radiological Museum of the Institute of Orthopaedics, London, have been reviewed and an attempt has been made to correlate the sclerosing lesions with the sclerotomes. The investigation was handicapped by paucity of films and clinical information, but in 19 cases the skeletal abnormalities were considered to correspond with a single sclerotome or part thereof. These studies were convincing when films of an affected hand or foot were available. In the remaining 11 cases multiple sclerotomes appeared to be involved and the clinical manifestations were correspondingly more severe. It is proposed that melorheostosis may be the late result of a segmental sensory nerve lesion, to account for its sclerotomal distribution. The association with linear scleroderma is discussed, since it has been suggested that these cutaneous lesions are related to the same nerve segment. Eight cases showed paraarticular ossification of soft tissues which may be related to involvement of a corresponding myotome.

Adult↗

PubMed1982

Melorheostosis of the axial skeleton with associated fibrolipomatous lesions.

Two patients with melorheostotic-like lesions of the axial skeleton are described. In each case adjacent soft tissue masses containing both fatty and fibrous tissues were evident. The presence of such soft tissue tumors as well as other soft tissue abnormalities in melorheostosis emphasizes that the disease should not be regarded as one confined to bone. The precise pathogenesis of the osseous and soft tissue abnormalities in melorheostosis remains obscure.

Adult↗

PubMed2002

Osteosarcoma arising in a femur with melorheostosis and osteopathia striata.

Osteopathia striata is an asymptomatic autosomal dominant or sporadically inherited disorder that causes dense striations at sites of endochondral bone formation, with a predilection for the metaphyses of long bones. Melorheostosis is a mixed sclerosing dysplasia with disturbance of both endochondral and intramembranous ossification, in which disordered intramembranous ossification dominates. It presents typical radiological changes of cortical hyperostosis distributed along a sclerotome with variable associated cutaneous and clinical features. Overlap syndromes including one or more of these diseases are described. We report a 44-year-old man with both melorheostosis and osteopathia striata who presented with pain secondary to superimposed osteosarcoma. In reporting this case we discuss the relationship between sclerosing dysplasia and either coexisting or complicating sarcoma.

Adult↗

PubMed2002

Thoracic cage extent of melorheostosis depicted by multislice CT.

The ribs and vertebrae are rarely affected by melorheostosis. We present a case of melorheostosis of the ribs and thoracic vertebrae, in which multislice computed tomography (CT) was useful in depicting its extent and defining its sclerotome distribution.

Adult↗

PubMed2002

Melorheostosis of the hand with complicating bony spur formation and bursal inflammation: diagnosis and treatment.

A 37-year old woman presented with intolerable pain associated with a slowly growing mass on the dorsum of the right hand. The radiographs were characteristic of melorheostosis involving the third and fourth metacarpals, with a cortical bony projection extending from the base of the third metacarpal eroding slightly into the fourth metacarpal, associated with bursal formation. Magnetic resonance imaging (MRI) demonstrated an inflamed bursal collection adjacent to the spur. This region corresponded to the patient's severe clinical, focal pain. To our knowledge, this is the first case report in which melorheostosis of the hand has been associated with bony spur formation and complicated by an inflamed bursa.

Adult↗

PubMed2000

Melorheostosis: case report with radiologic-pathologic correlation.

Melorheostosis is an unusual mesenchymal dysplasia, which commonly presents on radiographs as longitudinal bars of hyperostosis in osseous structures. We present a case of melorheostosis in the lower extremity of a 20-year-old woman for which detailed radiologic- pathologic correlation was achieved due to amputation of the involved limb.

Acetabulum↗

PubMed2001

Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.

A 28-year old woman presented with Léri's disease (melorheostosis) and the rare combination of complex vascular malformations and lymphatic anomalies. Multifocal melorheostosis was segmental and unilateral, located in the left axial and peripheral skeleton, fifth thoracic vertebral body, fifth rib. left upper limb and lumbosacral spine (third lumbar body to first sacral segment). Sacral involvement was associated with spinal canal stenosis. Additionally the patient had multiple nevi and had suffered from left hemiplegia since birth. Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. Death ensued due to respiratory failure.

Adult↗

PubMed1992

Soft tissue contractures from melorheostosis involving the upper extremity.

Melorheostosis is a rare disorder characterized by a linear hyperostosis of cortical bone, joint pain, stiffness, deformity, and limited motion. In the hand, the disease is often disabling because of progressive contractures of the joints and soft tissues involved. Two cases of melorheostosis involving the radial side of the hand and upper extremity are reported. Splinting or surgical release did not result in permanent correction of the soft tissue contractures. Retraining patients to use their unaffected hand should be considered early in the course of the disease.

Adult↗