From Big Medical Encyclopedia

MELOCHEOSTOSIS (melorheostosis; grech, melos an extremity, the member + rheo to flow + osteon a bone; synonym Leri's disease) — sharp consolidation of bone structure of one or several bones, most often upper or lower extremity. For the first time M. is described in 1922 by fr. neuropathologists A. Leri and A. Joanny. The disease, apparently, is connected with anomaly of bone formation.

Pathoanatomical changes are characterized by excess formation of bone substance.

Fig. 1. Macrodrug of a cut of a tibial bone at a melocheostosis: the dense bone stratifications of a cortical layer reminding flows of a candle are specified by shooters (above — a view from the party of a cut, below — a look outside).

Macroscopically patol, process has an appearance of a bone thickening (hyperostosis), a cut at localization on long tubular bones (femoral, tibial, humeral) as if flows down from top to down with formation of the characteristic beddings reminding flows of the burning candle (fig. 1).

Only single observations of microscopic examinations of bones at M. therefore its essence remains obscure are published.

Fig. 2. Microdrug of a bone at a melocheostosis: a chaotic arrangement and various size of osteocytes — 1 and channels of osteons (gaversovy channels) — 2; coloring hematoxylin-eosine; X 200.

Histologically in a zone of periosteal beddings the uneven size of channels of osteons (gaversovy channels) with the wrong formation of osteonny structures is found. Osteocytes in these sites have the different form and size and are distributed randomly in system of osteon (fig. 2). The new growth of bone substance, uneven arrangement of lines of pasting and the phenomena of a smooth resorption of a bone which are poorly expressed in subperiosteal departments is noted.

A. V. Rusakov carries M. to group of inborn fabric displaziya on the ground that the disease begins in the early childhood and localization patol more often, process in certain sites of bone system points to its communication with ugly development of a mesenchyma. In favor of the dysplastic nature of a disease also the discrepancy revealed microscopically between rates of an osteogenesis and the slowed-down rassasyvaniye of a bone tissue testifies. About the insufficient change of bone generation necessary for physiological resorptive modeling of bone body, also a large number of cracks of bone plates demonstrates. Cracks are formed in processing of drug, however their origin, undoubtedly, is connected with change physical. - chemical properties it is long not renewed bone.

A clinical picture

Patients are disturbed, as a rule, by the dull aches amplifying at night. Intensity of pains depends on load of the struck segment of an extremity. Pains are localized not only in the place of damage of an extremity, but can irradiate in other its segments, especially at a prelum of vascular and nervous educations. Uncertain heavy feelings, weaknesses of the affected extremity, an atrophy of her muscles are characteristic of patients of young age, the rigidity of one or several joints which is gradually progressing joins later.

In the started cases sites of a scleroderma, and also deformation of extremities of various degree are found according to bone defeats. Than the patient is more senior, that a wedge, a picture M. vyrazhenny. Due to the indistinct and uncertain symptom complex of M. began diseases usually it is not possible to establish or long time rheumatism or other diseases are mistakenly diagnosed.

Diagnosis it is possible only with the help rentgenol, researches.

Fig. 3. Roentgenograms of hands: at the left — is normal (for comparison), on the right — the 29-year-old patient with a melocheostosis (consolidation and a thickening of phalanxes of IV, V and partially the III fingers of the right brush on ulnarny edge; deviation of IV and especially the V finger in the ulnarny party).

On the roentgenogram the sites of sharply condensed structure located so that an impression about allegedly happened «running off» of osteosclerotic mass of a bone with above-located on the below-located departments is made are visible. Are surprised preferential long and short, and also ossicles of an extremity (fig. 3), are more rare shovels and haunch bones. Consolidation of structure is observed not throughout the affected bone, and on its certain site, preferential excentricly. In addition to a sklerozirovaniye of spongy substance of a bone, at M. the hyperostosis of the affected bones is quite often observed. In long and short tubular bones it is shown in the form of unilaterally the assimilated periosteal stratifications thickening and deforming a diaphysis. At long observation increase of extent and expressiveness sometimes is found osteosclerosis (see), hyperostosis (see) and deformations.

At differential diagnosis it is necessary to consider the following. Syphilitic (gummous) osteomyelitis (see) does not lead to a uniform sclerosis of a diaphysis and a metaphysis of tubular bones only of one extremity and never affects short spongy bones. Marble disease (see) it is never found in bones only of one extremity, affects almost all skeleton, often being followed by the accruing heavy anemia, it is frequent patol, changes and other complications. A fibrous dysplasia (see. Fibrous osteodysplasia ) in a phase of consolidation does not give a diffusion osteosclerosis; usually in nek-ry sites the razvolokneniye of bone structure is observed, and it is frequent even cystic changes. The fibrous dysplasia exclusively seldom can affect short spongy bones of extremities.

Treatment comes down to symptomatic actions, etiopatogenetichesky means it is not found.

Forecast for life favorable. Function of extremities can be broken in various degree.

See also Bone .

Bibliography M. V. wolves, Disease of bones at children, page 394, M., 1974; The Multivolume guide to pathological anatomy, under the editorship of A. I. Strukov, t. 5, page 197, 474, M., 1959; The Resident of Perm about in N. K. Sluchay of the isolated melocheostosis of a tibial bone, Arkh. patol., t. 23, No. 12, page 77, 1961; P about x l and N D. G. Meloreostoz, Vestn, rentgenol, and radio-gramophones, t. 9, century 4, page 292, 1931, bibliogr.; Leri A. and. J about a n n at, Une effection non ctecrite des os, hyperostose «en coulee» sur toute la longueur d’un membre on «melorheostose», Bull. Soc. med. Nbr. Paris, t. 46, p, 1141, 1922; R i t v o M. Bone and joint X-ray diagnosis, p. 106, L., 1955.

D. G. Rokhlin, H. G. Permyakov.