(osteodysplasia fibrosa; synonym: fib
a different dysplasia of bones, Likhteistayn's disease — Braytseva, Jaffe's dysplasia — Likhtenstayna) — the malformation of bone system, to-rogo is the cornerstone the disturbance of ossification leading to deformation of bones.
It is for the first time allocated in an independent nosological form in 1938 an amer. scientist L. Likhtenstayn. under the name «poliostotichesky fibrous dysplasia».
In a crust, time as most of researchers it is recognized that F. by the lake it is connected with disturbance of normal ossification of a skeleton in the period of embryonic development. According to T. P. Vinogradova (I960), T. the island treats group of processes, boundary with benign tumors. Ability to recur after removal and to be exposed to a malignancy confirms this point of view and allows to consider F. the lake as predop at a cholic state. According to M. V. Volkov (1974), among new growths and displaziya of a skeleton observed at children's age, T. the lake makes 8,5%.
According to number involved in patol. process of bones distinguish monoossalny and poliossal-ny forms F. lake Patol. changes arise more often and are more shown in jaws (see. Fibrous osteodysplasia of jaws), in proximal departments of extremities.
The lower extremities are surprised more often than upper. From flat bones at F. islands suffer preferential front departments of a calvaria and ileal bones. In long tubular bones process is, as a rule, localized in metaphyses from where extends to a diaphysis. The zone of an epiphyseal cartilage is a peculiar barrier for F. lake. In rare instances an epiphysis is surprised initially, finds secondary nonspecific changes in them more often — osteoporosis and deformation.
Pathological anatomy. The nature of pathoanatomical changes at F. the lake does not depend on a form of a disease. The struck departments of bones are deformed, thickened, long tubular bones are quite often bent. On a cut of bones find various size and a form the centers of the whitish dense fabric which is usually containing small bone inclusions. In some cases there is almost full substitution of a bone this fabric. Over the centers F. the plate of compact (cortical) substance is thinned by the lake, on certain sites is interrupted; in the thickness patol. fabrics are located the cysts containing transparent liquid.
At microscopic examination of the struck departments of bones the fibrous connecting fabric presented by collagenic, reticulin fibers and fibroblastopodobny cells is found, among to-rykh the primitive bone beams which are distributed irregularly (fig. 1) are located. Quite often peripheral departments of bone beams consist of ossiform fabric, less often beams entirely consist of this fabric. Sometimes the forming bone structures are extremely primitive and have an appearance of more or less isolated condensations of collagenic fibers without adjournment of salts of calcium in them. These structures consider as a premode of formation of bone beams. At different patients and in various centers F.
lake Rig*. 1. Mikronreparat of the site of fabric silt of the pathological center at a fibrous osteodysplasia: fibrous connecting fabric with single irregularly raise> l about e N ny m both p r at it and ti in N y m and to from a tna in m and beams (are specified by shooters); coloring hematoxylin-eosine, x 10 5.
the same patient and even in different sites of one center gistol. the picture can be unequal. It differs on the nature of bone structures, a quantitative ratio of these structures with fibrous fabric, morphology of cells and density of an arrangement of fibers. At nek-ry patients in patol. the centers or their sites more or less dense network of bone beams sometimes reminding a spongy osteoma (see) develops. Accumulations of osteoclasts occur among fibers of connecting fabric; occasionally osteoblmstoklastoma sites are found (see), accumulations of ksan-volume cells sometimes come to light (see the Xanthoma).
The clinical picture is various and depends on a form F. lake, localizations of process and rasprostra-
Fig. 2. The patient with defeat and characteristic deformation of the lower extremities at on-liossalnoy a fibrous osteodysplasia: proksimal
the ny department of the left hip has varus deformation, the big spit is lifted to the level of a body of an ileal bone; valgus deformation in the right knee joint.
nennost of bone defeats. Patients complain of pains during the walking which intensity amplifies during the progressing patol. process. Quite often first symptom of a disease are repeating patol. changes. The most frequent sign F. the lake — deformation of a bone from its scarcely noticeable curvature to a considerable bend of the whole segment of an extremity. Separate bones are exposed to characteristic deformation (fig. 2). At localization of the center F. the lake in the proximal end of a hip it takes a form of a shepherd's stick with a bend camber of a knaruzha, the big spit is lifted up to the level of a body of an ileal bone; varus deformation of a hip develops. Shins at their defeat are bent more often than a knaruzha and a kpereda. Humeral bones it is club-shaped are thickened in proximal departments. Defeat of pelvic bones is followed by deformation of a pelvic ring, vertebrae, the Rebbe oh, of a thorax. Damage of bones of a skull and their deformation cause asimmetryayu and a disfiguration of the person, secondary vision disorders and hearing, the heads-nys of pain, an exophthalmos (see). Deformations of bones are aggravated incorrectly accrete patol. changes. Almost constant symptom F. the lake of bones of the lower extremity is lameness (see), from easy limping before the expressed disturbance of gait. Functional inferiority of an extremity is followed by an atrophy of soft tissues, secondary deformations of joints, a backbone.
Poliossalny form F. the lake is often combined with patol. skin pigmentation (a spot of various size and a form of all shades of brown color), early puberty (see), a hyperthyroidism (see the Thyrotoxicosis). Bone changes, a xanthopathy and premature puberty at women — a triad of the signs known as a syndrome, or a disease, Albright (see the Pseudohypoparathyroidism). Progressing F. the lake comes to an end with puberty.
The diagnosis is established on the basis by a wedge, pictures and typical rentgenol. data. Leaders rentgenol. signs F. lakes are the center of depression and a zone of a diffusion enlightenment of a bone tissue. According to it it is accepted to distinguish two rentgenol. forms F. lake: focal and diffusion. The border between them, however, is not always clear, the mixed forms are possible.
At a focal form F. islands find the sites of a limited enlightenment of a bone tissue which are located eccentric in the field of cortical (compact) substance of a bone. Originally arising nodes are accurately delimited from each other by the skorlupoobrazny condensed strip of a bone tissue. In process of development of a disease the sizes of the centers increase; thinning of cortical substance is found, a cut gets uneven crescent or scalloped outlines. The bone is as if blown up from within, becomes hilly. Sometimes reveal at the same time several isolated centers F. lakes, to-rye are indistinctly delimited from each other, but nevertheless keep relative independence; at the same time the bone takes a coarse form (fig. 3). Its cortical substance sharply becomes thinner at the expense of an endosteal resorption. Diameter of a bone on a considerable extent is unevenly thickened. In nek-ry cases the centers F. lakes are defined in the form of separate nests-nykh of the educations scattered on a llinnik of a bone among not changed pla of the low-changed, generally sclerosed, bone tissue. The structure of the centers can be various. Often they have an appearance of homogeneous enlightenments, is more rare — chalk -
Fig. 3. The roentgenogram of a distal third of a shin of the patient with a focal form of a fibrous osteodysplasia of a tibial bone (a direct projection): chetno the delimited serious cellular defect of a bone tissue with the bast layer thinned over it.
koyacheisty, porous structure, certain sites of consolidation, calcification or ossification in the form of incorrectly created bone beams sometimes contain. Localization, the sizes and quantity of the centers change in the course of growth and development of the bone and joint device; the tendency to their merge, shift to the middle of a diaphysis is observed. At stabilization of a disease rentgenol. the picture a long time remains invariable.
At a diffusion form F. the island is surprised all thickness of a bone. Quite often various angular and arc-shaped deformations of bones are defined. Most often reveal varus deformation of a neck of a femur (fig. 4), varus or valgus deformation in a knee joint. In places of the largest tension of the bent bones zones of dystrophic reorganization (see Loozer of a zone) and patol can be defined. changes.
Characteristic patol. changes come to light in spongy substance and a marrowy cavity. A plate of cortical substance uneven, scalloped, thinned. The volume of a bone increases. Sites of homogeneous soft fibrous mass are displayed on the roentgenogram in the form of a uniform enlightenment. On this background of accumulation of ossiform fabric cause more dense shadows. Areas of dense ossiform fields with existence of punctulate elements of calcification and ossifika-
Ts1sh have very characteristic for F. the lake the structure reminding ground glass. In the centers F. lakes can come to light cystous cavities. Rentgenol. the research of an obn r has supper dynamics of their education: in the beginning increase in the sizes of the site of fibrosis, then its transition to focal osteoporosis and, at last, formation of a true cystous cavity. Along with an enlightenment of a bone tissue at F. lakes are observed sites of consolidation of a bone. They are located preferential subkortikalno, is frequent on all diaphysis, but is sometimes limited, in the form of local implementation of yazykopodobny strips. Feature rentgenol. signs of damage of bones of a skull the prevailing development of an osteosclerosis (see) and a hyperostosis is (see). Excess growth of a defective bone tissue is combined with reduction of a head cavity, narrowing of an eye-socket and other vital spaces of a skull.
Diagnosis of a poliossalny form F. the lake in most cases does not raise doubts. The differential diagnosis carry out with a chondrodysplasia (see), a parathyroid osteodystrophy (see), Hend's disease — Schueller — Krischena (see Hend — Schueller — Krischena a disease). Mo-noossalnuyu form F. lakes differentiate with dystrophic, tumoral, inflammatory processes in bones (a bone cyst, an osteoblastoclastoma, a tubercular osteitis).
Fig. 4. The roentgenogram of a femur (a direct projection) of the patient with a diffusion form of a fibrous osteodnsplaziya: considerable varuyeny deformation of proximal department of a femur is noted; the femur is thickened, has large cell structure with bone crossing points.
Treatment of patients with monoossalnop a form F. lake operational; all types of conservative treatment are inefficient. The indication to operation are pain, the expressed deformations, progressing of process, threat patol. change. An early operative measure (at the age of
3 — 4 years) improves the forecast, prevents development of deformations, shortening of an extremity. Depending on localization and prevalence of process make a subperiostal regional or segmented resection of the struck department of a bone with substitution of the formed defect a bone transplant (see. Bone plastics). Apply to plastics of defect auto-or allotransplants in the form of small, large or thin plates of cortical substance. At sharp deformation of femoral, tibial bones the alloplasty is combined with a corrective osteotomy (see) and bone fixing by a metal plate. During the shortening of an extremity lengthening of a bone with the help dis - the traction and compression device (see Distraktsionno-kompres-s and about and, N ye an appara of t y). Ortopediches to and e devices (see) and wearing special footwear (see Footwear, orthopedic footwear) in a complex with active rehabilitation therapy in the postoperative period (massage, to lay down. gymnastics) create conditions for normalization of a statics, promote bystry and correct reorganization of a bone.
At a poliossalny form F. lake treatment same. However plurality of defeat limits possibilities of use of operative measures.
The forecast for life in most cases favorable. However sometimes, both at poliossalny, and at monoossalny F. the lake, comes a malignancy with development of an osteosarcoma (see) or fibers about cap to ohms (see). The forecast concerning function of an extremity depends on a form of a disease and features of its current. Patients with a poliossalny form F. lakes shall be oriented to acquisition of the professions which are not connected with hard physical work, long standing, walking.
See also Fibrous osteodysplasia of jaws.
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JI. I. Samoylova; I. P. Korolyuk (rents),
V. S. Yagodovsky (stalemate. An).