CHONDROMATOSIS of KOSTYoY (chondromatosis ossium; Greek chondros a cartilage + - oma, omatos - f--osis; synonym: Ollye's disease, a dyschondroplasia, multiple enkhondromatoz) — the inborn general progressing disease of a skeleton, is shown by preferential unilateral disturbance of an endostosis; one of forms of a chondrodysplasia.
The combination of a chondromatosis of bones to a generalized angiomatosis, lipomatozy, a fibromatosis and a nevus of skin makes a complex of the signs characteristic of a syndrome of Maffuchchi (see Maffuchchi a syndrome).
One of the first kliniko-rentgenol. the picture of a chondromatosis of bones was described by the fr. surgeon Ollye (L. X. E. Oilier) in 1899. He allocated this disease as a nosological form and offered the term «dyschondroplasia».
The etiology is unknown; there are various theories of emergence of a chondromatosis of bones, in particular the theory of defeat of primary laying of a skeleton arising in
Fig. 2. The roentgenogram of brushes at a chondromatosis (a direct projection): phalanxes and
metacarpal bones are shortened, their epimetafizarny ends are sharply thickened, as if blown up at the expense of the numerous hondromatozny centers.
4 — the 5th week of pre-natal development, as a result the cut is slowed down and ossification of an embryonal cartilage is perverted.
The centers of a cartilaginous dysplasia arise in bones of a mesenchymal origin, are located in the most quickly growing metaphyses of long tubular bones more often: in proximal department of a humeral bone, a distal metaphysis of beam and elbow bones, the proximal and distal ends of femoral and tibial bones. Seldom damage of vertebras and only in isolated cases — bones of a base of skull meets. With age the centers increase in sizes, are exposed to calcification and ossification. They deform a bone, destroy compact (cortical) substance and a periosteum, burgeon in soft tissues, sometimes strike an epiphysis that leads to disturbance of congruence of joint surfaces. Defeat of active regions of growth of long tubular bones is followed by deformation and shortening of extremities.
Macroscopically the centers of a chondromatosis look as roundish knotty cartilaginous formations of gray-white or bluish color with separate opaque and yellow impregnations — sites of calcification and ossification. The centers fill a marrowy cavity or are located in the thickness of spongy substance of an epiphysis and metaphyses of bones (fig. 1). Compact substance over the hondromatozny centers is thinned, quite often destroyed. At microscopic examination hondromatozny fabric usually has a lobular structure. Cartilaginous cells of various form are located
in interstitial substance randomly with either separate accumulations or continuous weight, sometimes develop in peculiar beams, races -
Fig. 1. Macrodrug of the proximal end of a humeral bone at a chondromatosis (a sagittal cut):
the center of a chondromatosis in spongy substance of a bone is visible white color, over the Crimea sharp thinning of compact substance is noted.
the segments which are gone from the center. Ossification is usually more expressed on the periphery of the centers of a chondromatosis.
In nek-ry cases the disease is revealed since the birth, but usually — since that period when the child starts walking; it is shown more often in the form of lameness (see), and then — deformations of one of the lower extremities. At a functional load small morbidity in zones patol can be noted. changes. Sometimes the pathological change (see) happens the first symptom of a disease. The movements in joints are limited slightly; at adults the phenomena of arthrosis, a moderate muscular atrophy develop.
Shortening of the lower extremity in combination with valgus or varus deformations of bones and joints is followed by disturbance of its oporosposobnost (see fig. 16 to St. Bone, t. 11, Art. 457). With the slowed-down growth of one of pair bones develop beam or elbow talipomanus (see).
In diagnosis of a chondromatosis of bones plays an important role rentgenol. research. At the same time the X-ray analysis of all departments of a skeleton is obligatory. The affected tubular bones are shortened, their epimetafizarny ends are thickened, as if blown up at the expense of the numerous hondromatozny centers (fig. 2). In metaphyses sites of an enlightenment of heterogeneous character (fig. 3) with tape-like consolidations of a bone tissue are visible, to-rye fanlikely disperse towards an epiphysis. At the expressed chondromatosis the extensive uneven enlightenments (see Loozer of a zone) occupying all affected bone (fig. 4) are defined. In it the certain chaotically located sites of a bone tissue and the melkokrapchaty centers of calcifications are visible. Cortical substance of a bone over the centers of defeat, especially at their excentric arrangement, is thinned or destroyed and hangs in the form of a visor. At defeat of pelvic bones, mainly in their peripheral departments, the multiple oval, triangular or polygonal enlightenments crossed by rough accumulations of a bone tissue are found.
Differential diagnosis is carried out with other forms of a chondrodysplasia (see). At the same time consider hl. obr. characteristic kliniko-rentgenol. picture and results of microscopic examination of operational material.
Treatment at moderately expressed process is based on use of orthopedic devices, napr, orthopedic footwear (see), orthoses (see) that allows to prevent development of deformations of a skeleton. At the expressed deformation with dysfunction of an extremity, and also bystry development of process an operative measure is shown. Deformation and shortening of an extremity are eliminated by an osteoclasis (see the Osteotomy) with imposing of the distrak-tsionno-compression device (see), and also a distraktsionny epn-fizeoliz (see Changes) or osteotomies (see). At quickly I accrue -
Fig. 3. The roentgenogram of forearms and proximal departments of brushes at a chondromatosis (a direct projection): deformation of bones of forearms with a thickening of distal metaepiphyseal departments where are visible неод^-нородные sites of enlightenments an arrow it is specified a zone of reorganization of a bone (Loozer's zone).
Fig. 4. The roentgenogram of a hip joint and upper third of a hip at a chondromatosis (a direct projection): extensive uneven enlightenments (the centers of a chondromatosis) occupying all metadiafi-zarny part of a femur; cortical substance of a bone over the centers of defeat is sharply thinned.
Russian cabbage soup of deformation in the field of the hondro-matozny center the regional resection or a corrective osteotomy with removal a chondroma-toznoy of fabric and bone plastics is shown (see).
At damage of bones of a brush and foot the early operational treatment preventing ugly deformations is shown: a scraping,
a regional or segmented resection with bone plastics, sometimes — a corrective osteotomy.
The forecast for life favorable. The forecast in the functional relation depends from a wedge, courses of a disease and often it is adverse in connection with the continuing increase in deformations. Exceptional cases of self-healing are described. The malignancy of the centers of defeat is observed seldom.
Dispensary observation is shown to patients (see Medical examination).
Bibliography: Andrianovv. JI. and M about r-@ at V. A N. Operational treatment of deformations of extremities at children at Olye's disease, in book: Aktualn. vopr. travmat. and the orthoitem, under the editorship of M. V. Volkov, century. And, page 70, M., 1975; In and N about r and d about in and T. P. Tumors of bones, page 240, M., 1973; In about l to about in M. V. Diseases of bones at children, page 249, M., 1974; Volkovm.V., etc. Hereditary general diseases of a skeleton, M., 19 82; To about with and N with to and I am N. S. Disturbances of development of the bone and joint device, L., 1966; The Multivolume guide to orthopedics and traumatology, under the editorship of N. of the Item No-vachenko, t. 2, page 516, M., 1968; The Multivolume guide to pathological anatomy, under the editorship of A. I. Strukov, t. 5, page 377, M., 195'9; R e y N e r S. A. X-ray
diagnosis of diseases of bones and joints, book 1, page 432, book 2, page 554, M., 1964;
Aeger ter E. E. a. Kirkpatrick J. A. Orthopedic diseases, p. 108, Philadelphia a. o., 1968; L e w i s R. J. a. Ketch an in A. S. Maffucci's syndrome, functional and neoplastic significance, J. Bone Jt Surg., y. 55-A, p. 1465, 1973; About llier L. De la dyschondroplasia, Bull. Soc. chir. Lyon (1899), t. 3, p. 22, 1900; S with h a j o-w i with z F. Tumors and tumorlike lesions of bone and joints, p. 109, 117, N. Y. a. o., 1981. Ya. O. Oll; I. P. Korolyuk (rents.),
S. I. Lipkin (stalemate. An.).