From Big Medical Encyclopedia

HERUVYZM (synonym: a family fibrous dysplasia, a family polycystic disease) — the rare hereditary disease which is shown a malformation of a facial skeleton, preferential top and bottom jaws. A peculiar appearance of patients is characteristic, the person to-rykh becomes roundish and reminds images of persons of angels that gave the grounds to W. A. Jones who for the first time described this disease in 1933 to call it a heru-vizm (from the word «cherub»).

Heruvizm is a heredo-familial disease, is inherited both in the area of mother, and in the area of the father.

At a heruvizm disturbance of formation of mesodermal structures, and also involvement in process of derivatives of an ectoderm is noted. In jaws there are centers of an unripe bone tissue with the expressed processes of an osteoklastichesky resorption and bone formation. Period of the most intensive growth patol. the centers it is the share of age

from 3 — 4 to 11 — 12 years. In the active period of a disease the affected bone is hilly, a surface its uneven, is stained brown or crimson-brown color with a cyanochroic shade. Along with sites of thinning and lack of compact substance there are zones of its thickening and a sklerozirovaniye. From compact substance easily separate soft patol. mass of uneven gray-yellow-brown color.

Microscopically patol. the center is presented by cellular and fibrous fabric, edges is considerably condensed, is less often edematous. Accumulations of huge multinucleate cells like osteoclasts occur among wide layers of cellular and fibrous fabric (see the Bone), the quantity to-rykh increases during the active period of a disease. The small, freely lying accumulations of erythrocytes and small deposits of a gemosidery meet (see the Hemosiderosis). In the central departments patol. the centers there are small focuses of imperfect bone formation (see Bone formation imperfect) in the form of small deposits of osteoid, in Krom osteocytes are randomly scattered.

With approach of puberty process patol. reorganizations in jaws calms down, the tendency to the intensive osteogenesis which is coming to an end with formation of a bone of normal structure is noted.

The first symptoms of a disease usually appear at the child between 1 — 3 years. Disease is wavy, the periods of relative calm alternate with the periods of rapid growth patol. the centers, leading to deformation of jaws. Symmetric defeat top and bottom jaws is characteristic of a heruvizm. The face of the patient due to bilateral protrusions in perednebokovy departments takes as if a mumpish, masklike form, nasolabial folds are maleficiated, the mimicry is broken (tsvetn. tab., Art. 208, fig. 15). The eye is turned up why the white strip of a sclera in a lower eyelid is sometimes visible. Anomalies of formation and an arrangement of teeth (see), a delay their eruptions (retention) are characteristic of a heruvizm. Usually these changes are irreversible. Nodes are almost always increased regional limf. By 14 — 17 years the face of the patient, as a rule, accepts usual outlines. At nek-ry patients rugged features due to increase in a body and branches of a mandible are saved. The unilateral exophthalmos (see) with decrease in visual acuity can be observed.

Diagnosis is based on identification of characteristic kliniko-rents-genol. signs. At a palpation of a jaw are, as a rule, painless. A consistence palpated patol. educations it is various depending on a stage of process and age of the patient. At children these educations are more elastic and pliable, teenagers have a consistence patol. educations becomes more dense.

Characteristic rentgenol. signs are symmetry and uniformity of defeats on both sides. Changes on a mandible originally arise in the field of its corner and branches. Gradually process extends to a body of a jaw (tsvetn. tab., Art. 208, fig. 16). Almost always the coronal shoot is involved in process. Multiple accurate cellular cystic educations alternate with rough shapeless calciphied bone trabeculas.

Rentgenol. the picture of damage of an upper jaw is less characteristic. Patol. the shadow is projected on Highmore's (maxillary, T.) a bosom, edges it is partially darkened. The cellular structure of a bone is well-marked, and cells have various size and the form. Along with it quite large homogeneous centers of the increased density are defined. In any site upper or a mandible the impacted teeth can meet (tsvetn. the tab., Art. 208» fig. 17 — 19).

10 — 15 years in the area patol are aged more senior. the centers process of ossification radiological begins to come to light, to-ry comes to an end with recovery of structure of a bone. In the outcome patol. process along with moderate deformation of a body of the mandible the indistinct cystic centers of destruction radiological are defined, is more often in the field of branches. Sometimes the phenomena of a focal or diffusion sclerosis up to formation of zones of continuous petrification prevail.

The differential diagnosis of a heruvizm is rather simple. From other opukholepodobny and tumoral diseases of a facial skeleton it is distinguished by heredo-familial character, symmetry of damage of jaws, anomalies of teeth, characteristic rentgenol. picture.

Treatment is begun if necessary with the orthodontic help. At early loss of teeth, an edentia, retention make prosthetics. In some cases at sharply expressed deformations distorting features of the patient an operative measure — ex-kokhleation (see) patol is shown. centers.

The forecast for life favorable.

Bibliography: Kolesov A. A. About

a so-called heruvizm. (To kinds of a fibrous dysplasia of jaws), Stomatology, No. 6, page 35, 1961;

Jo nes W. A. Familial multilocular cystic disease of jaws, Amer. J. Cancer, v. 17, p. 946, 1933. V. V. Roginsky.