Melorheostosis is a very rare sclerosing bone disease with pain, joint stiffness, and limitation of motion. Its characteristic on radiography is a dense bone formation along the side of the bone, resembling the flow of candle grease. Various conservative or surgical methods have been practiced in treating pain and deformities. This is a report on the successful treatment result of six cases of melotheostosis, involving the metatarsal, metacarpal, clavicle, and tibia, which weretreated by conservative and operative treatment methods.
Arthralgia ; Bone Diseases ; Clavicle ; Congenital Abnormalities ; Melorheostosis* ; Metatarsal Bones ; Osteogenesis ; Radiography ; Tibia

Adult ; Foot Diseases ; diagnostic imaging ; Humans ; Male ; Melorheostosis ; diagnostic imaging ; Radiography ; X-Rays

Melorheostosis is a rare disease, belonging to the sclerotic bone dysplasia group. Initially described by Leri and Joanny in 1922, its etiology remains unknown. Onset is usually insidious, with deformity of the extremity, pain, limb stiffness, and limitation of motion in the joints. The typical radiographic appearance consists of irregular hyperostotic changes of the cortex, resembling melted wax dripping down one side of a candle. Treatment is usually symptomatic and conservative; however, conservative treatment is unsatisfactory due to functional issues when involving the distal extremity. We report on two cases of melorheostosis with synovial chondromatosis of the foot treated by mass excision.
Ankle ; Bone Diseases, Developmental ; Chondromatosis, Synovial* ; Congenital Abnormalities ; Extremities ; Foot* ; Joints ; Melorheostosis* ; Rare Diseases

Melorheostosis is a rare, progressive bone disease accompanied by hyperostosis and soft tissue fibrosis. While affected adults present with contracture and pain, children present with limb length discrepancy and deformity. We report the case of a 20-year-old woman with melorheostosis since childhood who presented with right hand deformity and numbness. Radiographs showed not only a combination of dense sclerosis and opacities, but also the classic 'flowing candle wax' appearance. Radiography can be used to identify melorheostosis, thus preventing unnecessary bone biopsies. Carpal tunnel release revealed the presence of a thickened flexor retinaculum and a degenerated median nerve distal to the retinaculum, but did not show hyperostosis. This case highlights the role of nerve decompression in melorheostosis and the importance of early identification of the disease to prevent unnecessary bone biopsies.
Carpal Tunnel Syndrome ; complications ; diagnostic imaging ; surgery ; Diagnosis, Differential ; Female ; Fibrosis ; pathology ; Hand ; pathology ; Hand Deformities ; diagnostic imaging ; surgery ; Humans ; Hypesthesia ; Median Nerve ; surgery ; Melorheostosis ; complications ; diagnostic imaging ; surgery ; Radiography ; Sclerosis ; diagnostic imaging ; Young Adult

We report a 56-year-old female with symptomatic protrusion of the bony lesion in the trapezium. Excision and biopsy of the bony lesion revealed thickened and sclerotic bony trabecula with adjacent zone of fibrocartilage, which is comparable with melorheostosis. This lesion with unique radiologic and histologic findings may be important to differentiate with other bony lesions such as myositis ossifications and osteosarcoma.
Biopsy ; Female ; Fibrocartilage ; Humans ; Melorheostosis* ; Middle Aged ; Myositis ; Osteosarcoma

Melorheostosis is a rare disorder characterized by irregular, flowing hyperostosis in long bones, commonly described on radiographs as wax flowing down a candle. In addition to bony sclerosis, cutaneous manifestations overlying the involved bones have been reported including linear scleroderma, neurofibromatosis, and vascular and lymphatic malformations. Unilateral nevoid telangiectasia (UNT) is a rare primarily cutaneous condition characterized by linearly arranged small dilated blood vessels in dermatomal or Blaschkoid patterns on the skin. Here, we present the case of a nine-year-old Korean male with UNT associated with ipsilateral melorheostosis.
Blood Vessels ; Humans ; Hyperostosis ; Male ; Melorheostosis ; Neurofibromatoses ; Scleroderma, Localized ; Sclerosis ; Skin ; Telangiectasis

Melorheostosis usually affects long bones of the lower limbs, and the hand is a relatively uncommon site of presentation. This disease is diagnosed by its characteristic X-ray finding and is confirmed by histololgic examination. Recently, Magnetic Resonance (MR) images have been used to evaluate disease activity and to understand soft tissue pathoanatomy in melorheostosis. The authors evaluated the activity of the melorheostosis indirectly with MR imaging and achieved a good symptomatic result with conservative treatments.
Hand* ; Lower Extremity ; Magnetic Resonance Imaging ; Melorheostosis*

PURPOSE: To evaluate the usefulness of various radiographic imaging modalities in the diagnosis and characterization of melorheostosis. MATERIALS AND METHODS: We retrospectively evaluated the plain film (n=8), computed tomographic (CT) imaging (n=5) and magnetic resonance (MR) imaging (n=5) findings of eight patients with melorheostosis diagnosed by bone biopsy (n=4) and characteristic radiographic findings (n=8). MR images were obtained with a 1.5-T scanner focused on the region of maximal radiographic abnormality. Pulse sequences include T1-weighted SE, T2-weighted fast SE (n=5) and postcontrast imaging (n=4). In order to define subtle enhancement of the lesions, subtraction MR images were obtained in one case. Imaging findings were analyzed with particular emphasis on the distribution of lesions along the sclerotome, differential radiographic findings between diaphyseal and metaepiphyseal lesions of the long bones, as seen on plain radiographs, and the density and signal characteristics of hyperostotic, lesions, as seen on CT and MR images. RESULTS: Characteristic distribution along the sclerotome was identified in five of eight cases mainly along C6 and 7 (n=2) and L3, 4 and 5 (n=3) sclerotomes. In diaphyseal melorherostosis (8/8), a characteristic finding, i.e., a wax flowing down from the candle, was identified on plain radiographs. In all three patients with metaepiphyseal melorheostosis (3/8), multiple round or oval hyperostotic lesions were seen in the epiphysis and metaphysis of the long bones. On CT, the marrow cavity was partly obliterated by hyperostotic lesions in all five patients with endosteal hyperostosis. Among these, central ground glass opacity with a sclerotic rim was seen in three patients. Periosteal hyperostosis was seen in two of five cases, being visualized as irregular excrescences in the periosteal region and surrounding soft tissue. Individual hyperostosis was visualized as hypointense on T1-weighted images and as a hyperintense center with a surrounding hypointense rim on T2-weighted images (5/5). On postcontrast images, central enhancement was noted in all four cases. In one of these, in which the degree of central enhancement was subtle, subtraction images (postcontrast SE- precontrast SE) also revealed a central signal increment. Central enhancement corresponded to the hyperintense center seen on T2-weighted images (4/4) and the ground-glass opacity seen on CT (2/2). CONCLUSION: Radiographic imaging plays a crucial role in the diagnosis of melorheostosis. The future role of gadolinium-enhanced MR imaging in the characterization of the lesion may be important though further evaluation and pathologic correlation is required.
Biopsy ; Bone Marrow ; Diagnosis ; Epiphyses ; Glass ; Humans ; Hyperostosis ; Magnetic Resonance Imaging* ; Melorheostosis* ; Retrospective Studies

Melorheostosis is a rare disease entity of bone with an etiology and pathogenesis. It cause pain and stiffness in an affected limb, and dense bone formation along the side of bone resembles the flow of candle dripping. Two cases of the disease involving left hand and wrist, and right lower leg are reported with the review of literature.
Extremities ; Hand ; Hyperostosis ; Leg ; Melorheostosis ; Osteogenesis ; Rare Diseases ; Wrist

Melorheostosis is a rare entity while causes pain and stiffness in a limb and has an unknown etiology. It is characterized by roentgenographic appearance of melting wax dripping down one side of a candle along the major axis of long bone. The present case represents a melorheostosis of the left tibia accompanied by synovial chondromatosis in the left knee. Good result was obtained by arthrotomy of the left knee and removal of the bony masses in the joint.
Chondromatosis, Synovial ; Extremities ; Freezing ; Joints ; Knee ; Melorheostosis ; Tibia