FERBANK DISEASE (Th. Fairbanks sovr. English radiologist; a synonym a multiple epiphyseal dysplasia) — the general disease of a skeleton from group of epiphyseal displaziya caused by the slowed-down and wrong formation of an epiphysis.
It is for the first time described in 1947. Fer-bank, to-ry expressed opinion on the inborn nature of a disease. This point of view in a crust, time admits almost all researchers who are engaged in studying F.; most of them indicates the hereditary and family nature of a disease. Families, in to-rykh F are described. passes from father to son; the analysis of family trees testifies about auto - somno - dominant type of its inheritance.
Pathogeny F. finally it is not found out. Defect of ossification of an epiphysis is the cornerstone of a disease (see the Bone). Late emergence and fragmentation of ossification centers in the field of an epiphysis of long tubular bones, their slowed down and maldevelopment, premature merge of an epiphysis and diaphysis lead to sharply expressed changes of a bone skeleton that is especially accurately expressed in the field of hip and knee joints. Over time the head of a femur gets a fungoid form, a distal epiphysis of femurs is flattened, an epiphysis of tibial, humeral bones sharply changes, fragmentation of a patella, its lobation, quite often dislocation comes to light. It results in incongruence of joint surfaces, a prematurity of dystrophic changes, first of all in hip, knee joints, a backbone.
Morfol. data at F. are presented by single observations. The joint cartilage in the field of an epiphysis has a hyaline structure, but in surface layers it is partially replaced with fibrous connecting fabric with inclusions of a cartilage. Epimetafizarny departments of change are expressed by an osteoporosis with formation of numerous lines of reorganization, thinning and an atrophy of bone beams and a subchondral bone plate, disturbance of its integrity on many sites, dystrophic changes of an epiphyseal cartilage.
Wedge, signs F. appear usually when the child starts walking, sometimes at later age. The main complaints — bystry fatigue and pains during the walking in joints of the lower extremities, lameness, disturbance of gait (the shaken gait). By 5 — 7 years low-tallness of patients due to shortening of the lower extremities with a normal length of a trunk is defined, varus or valgus deformations in knee and ankle joints develop, flexion contractures (see) in joints of top and bottom extremities appear. With increase in load of eiifiza dystrophic changes of a cartilage accrue that leads to the expressed pain syndrome and restriction of movements in the affected joints, an atrophy of muscles (see the Atrophy muscular), to a distortion of a basin (see), to a rachiocampsis (see).
Rentgenol. picture F. it is very characteristic. Typical changes are more often observed in tazobedren-
by Fig. Roentgenograms of a basin (a) and knee joints (b) of the child with a disease of Ferbank (a direct projection): epiphysis of femoral
and tibial bones is reduced in sizes, their height is reduced, pulled joint surfaces.
ny and knee joints. An epiphysis of bones is reduced in sizes, especially in the diameter; their basis is much shorter than an epiphyseal cartilage (fig., a); the distal ends of a diaphysis of femurs are flattened, an intercondyloid pole superficial, flat (fig., b).
Diagnosis F. the wedge, currents and typical rentgenol is based on idiosyncrasies. changes. The differential diagnosis should be carried out with Morkio's disease (see Morkio a disease), an osteochondropathy of a head of a femur (see Pertes a disease), an achondroplasia (see the Chondrodysplasia), an osteopoikilosis (see).
Treatment of hl. obr. conservative; it is directed to elimination of a pain syndrome, increase in amplitude of movements and the prevention of contractures in the affected joints, improvement of gait of patients. Lech. gymnastics (see. Physiotherapy exercises) and massage (see) promote improvement of a trophicity and tone of muscles; special exercises provide improvement of motive function of the affected joints. For the purpose of unloading of a joint, elimination of contractures and vicious provisions apply corrective laying. The dignity is of great importance. - hens. treatment, the best results to-rogo are observed at children 5 — 7-year age. Use of a balneoterapiya (see) and physical therapy (see), massage to lay down. gymnastics with unloading promotes increase in volume of movements in joints, to disappearance of pains, improvement of gait.
Operational treatment is applied when there are permanent statiko-functional disturbances (deformations, contractures or ankiloza in vicious situation). At bilateral defeat more defective joint in the functional relation or a segment of an extremity operate first of all. Corrective osteotomies (see) lower extremities at various levels and operation for congenital dislocation of a patella are most widespread (see). Operations on a hip joint almost do not make since they come to an end with an ankilozi-rovaniye of a joint. The patient 16 years are more senior endoprosthesis replacement is shown (see) hip joint. At any kind of operational treatment at patients F. the immobilization shall be minimum since it sharply limits the movements not only in operated, but also in not - the operated extremity.
At poorly expressed a wedge, signs when deformation of bones and joints does not lead to permanent statiko-functional disturbances, the forecast concerning function of extremities favorable. At the progressing disease sharply expressed combined deformations of joints lead to considerable functional frustration and disability of patients. Patients F. shall be oriented to acquisition of the professions which are not connected with long walking and standing, hard physical work.
Bibliography: M. V wolves. Diseases
of bones at children, page 327, M., 1974; In about M. V. l-kov, etc. Hereditary general diseases of a skeleton, page 63, M., 1982; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, book 1, page 416, M., 1964; F an i of -
b a n k T. Dysplasia epiphysialis multiplex, Brit. J. Surg., v. 34, p. 225, 1947; Rubin P. Dynamic classification of bone dysplasias, p. 86, Chicago, 1964; Spranger J. The epiphyseal dysplasias, Clin. Ortliop., v. 114, p. 46, 1976.
L. I. Samoylova.