Abstract
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 distribution 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 sclerodermal 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 para-articular ossification of soft tissues which may be related to involvement of a corresponding myotome.
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References
Campbell, C.J., Papademetriou, T., Bonfiglio, M.: Melorheostosis. J. Bone Joint Surg. [Am] 50, 1281 (1968)
Cunningham, D.J.: Manual of practical anatomy. 14th edition. G.J. Romanes (Ed.) London: Oxford University Press 1976
Dillehunt, R.B., Chuinard, E.G.: Melorheostosis Léri. A case report. J. Bone Joint Surg. 18, 991 (1936)
Henkel, L., Willert, H.-G.: Dysmelia: A classification and a pattern of malformation in a group of congenital defects of the limbs. J. Bone Joint Surg. [Br] 51, 399 (1969)
Inman, V.T., Saunders, J.B. de C.: Referred pain from skeletal structures. J. Nerv. Ment. Dis. 99, 660 (1944)
Léri, A., Joanny, J.: Une affection non décrite des os. Hyperostose ‘en coulée’ sur toute la longeur d'un membre ou ‘mélorhéostose’. Bull. Mém. Soc. Méd. Hôp. Paris 46, 1141 (1922)
McCredie, J.: Thalidomide and congenital Charcot's joints. Lancet 2, 1058 (1973)
McCredie, J.: Embryonic neuropathy: A hypothesis of neural crest injury as the pathogenesis of congenital malformations. Med. J. Aust. 1, 159 (1974)
McCredie, J.: Segmental embryonic peripheral neuropathy. Pediatr. Radiol. 3, 163 (1975)
McCredie, J.: Neural crest defects: a neuroanatomic basis for classification of multiple malformations related to phocomelia. J. Neurol. Sci. 28, 373 (1976)
McCredie, J.: Sclerotome subtraction: A radiologic interpretation of reduction deformities of the limbs. Birth Defects, XIII, 3 D, 65 (1977)
Morris, J.M., Samilson, R.L., Corley, C.L.: Melorheostosis. Review of the literature and report of an interesting case with a nineteen-year follow-up. J. Bone Joint Surg. [Am] 45, 1191 (1963)
Muller, S.A., Henderson, E.D.: Melorheostosis with linear scleroderma. Arch. Dermatol. 88, 142 (1963)
Murray, R.O.: Melorheostosis associated with congenital arterio-venous aneurysms. Proc. R. Soc. Med. 44, 473 (1950)
Patrick, J.H.: Melorheostosis associated with arterio-venous aneurysm of the left arm and trunk. J. Bone Joint Surg. [Br] 51, 126 (1969)
Soffa, D.J., Sire, D.J., Dodson, J.H.: Melorheostosis with linear sclerodermatous skin changes. Radiology 114, 577 (1975)
Stilwell, D.L.: The nerve supply of the vertebral column and its associated structures in the monkey. Anat. Rec. 125, 139 (1956)
Thompson, N.M., Allen, C.E.L., Andrews, G.S., Gillwald, F.N.: Scleroderma and melorheostosis. Report of a case. J. Bone Joint Surg. [Br] 33, 430 (1951)
Wagers, L.T., Young, A.W., Ryan, S.F.: Linear melorheostotic scleroderma. Br. J. Dermatol. 86, 297 (1972)
Zimmer, P.: Über einen Fall einer eigenartigen seltenen Knochenerkrankung. Osteopathia hyperostotica-Melorheostose. Beitr. Klin. Chir. 140, 75 (1927)
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Murray, R.O., McCredie, J. Melorheostosis and the sclerotomes: A radiological correlation. Skeletal Radiol. 4, 57–71 (1979). https://doi.org/10.1007/BF00349329
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DOI: https://doi.org/10.1007/BF00349329