Artrogripoz is a congenital disease of musculoskeletal system which is characterized by formation of contractures of a set of joints, damage of neurons of a spinal cord with primary atrophy of muscular structures. The clinical picture consists of deformation of articulate surfaces with restriction of volume of movements, hypotonias of muscles at safety at children of normal I.Q. and lack of damage of internals. For specification of the diagnosis use a X-ray analysis, KT and MPT. Treatment assumes an immobilization of the affected joints in functional situation, favorable to them, physical therapy, in hard cases ‒ application of surgical techniques.
For the first time pathology was in detail described in 1927 by the domestic surgeon E. Yu. Osten-Saken, but the term "artrogripoz" was offered by the American orthopedic surgeon M. Stern in 1923. Literally the name of a disease is translated as "a curve joint". In pediatrics meets frequency from 1:3000 to 1:56000 newborns; incidence is identical among both floors. Artrogripoz, or a syndrome of multiple congenital contractures, usually comes to light prenatalno within ultrasonic screening of a fruit or in the first days after the birth at survey of the newborn neonatology. For the purpose of prevention of invalidiziruyushchy consequences of a disease children from early age need intensive physical rehabilitation.
Reasons of an artrogripoz
Today more than one hundred reasons which promote formation of a disease are called, but main of them it is not allocated. It is authentically known what develops on 4-5 week of pre-natal development and is not descended (except a disteel form). Carry the following to possible causal factors:
- Teratogennye. Have destructive influence on the formed structures of a fruit. This group includes the use of the pregnant woman of drugs, alcoholic products and some drugs (for example, tsitostatik), ionizing radiation, toxic chemicals, infectious pathologies (a rubella, a Cytomegaloviral infection, toxoplasmosis) and others.
- Maternal. Are connected with features of a maternal organism. Include anomalies of anatomical structure of a uterus (a dvurogost, doubling, a hypoplasia), fetoplatsentarny insufficiency, existence of serious chronic illnesses (system red a wolf cub, diabetes, dekompensirovanny heart diseases) and other.
- Oligogidramnion. At the present stage many authors take lack of water for the main version of development of an artrogripoz. The small amount of amniotic waters causes lack of normal movements of a fruit owing to what joints are fixed in the certain compelled situation.
Pathogenetic mechanisms of an artrogripoz are studied not up to the end. In this respect there are several opinions. The Miogenny theory assumes primary degenerate defeat of muscular structures with further change of an articulate and copular complex and various parts of the nervous system. The Nevrogenny hypothesis says that in the prenatal period because of influence of set of causal factors there is a damage of motive ways of a spinal cord, and then formation of violations in the zones innervated by them. The Artrogenny version connects congenital contractures with initial involvement in a pathological complex of the capsular and copular device, an atrophy of surrounding muscles and a secondary miodegeneration.
Pathoanatomical opening indicates presence of changes in muscles of hypoplastic character. Their physiological points of an attachment are kept, but fibers settle down atypically because of the compelled extremity arrangement. The capsular device at an artrogripoza holds the surfaces of joints in hard contact, copular structures are shortened. Tubular bones are underdeveloped, atrofichna. Practically at each part of the nervous system there are degenerate processes.
By number of the affected joints and on the basis of the general condition of the patient it is classified on severity - easy, average and heavy. Separately distinguish types of contractures which happen taking away, rotational, razgibatelny, bringing, sgibatelny and combined. The international classification means division of a disease into the following forms:
- Generalized. Meets in 54% of cases. The disease can affect all groups of joints, including also such rare localizations as temporal and mandibular, grudino-akromialny, grudino-clavicular.
- With injury of the lower extremities. Makes 30% of total number of pathology. Defeats take foot joints, and also coxofemoral, talocrural, knee.
- With injury of the top extremities. It is observed in 5% of cases. Luchezapyastny, elbow joints and joints of brushes are surprised more often.
- Disteel. The number of sick children reaches 11%. Has about 9 subspecies among which there is a digital dismorfizm, a congenital arakhnodaktiliya and Gordon's syndromes, multiple pterigium, Freeman-Sheldon, a trismus-psevdokamptodaktiliya, etc.
Symptoms of an artrogripoz
The characteristic symptomatology consists of existence of contractures which have congenital character, and the muscular atrophy or a hypotrophy giving to patients the mass of an inconvenience. Defeats at an artrogripoza symmetric are also not inclined to progressing, only the recurrence of deformations after primary successful treatment is possible. In many cases the disease occupies the top departments of a trunk. A typical picture - hands are given also rotirovana inside, pronirovana forearms, elbows are unbent, brushes are bent towards palms. Injuries of the lower extremities often proceed with a clubfoot and incomplete dislocations of coxofemoral joints.
Artrogripoz is also combined with more rare manifestations - skin sindaktiliya (unions) of fingers, amniotic banners, gemangioma and teleangiektaziya, retractions of skin over the injured joints, sky crevices, heart diseases and to the meningomiyelotsela. In 10% of cases the combined vascular malformation located in a head or spinal cord are found. Patients are subject to frequent respiratory diseases. Important and positive feature is existence of the mental capacities corresponding to age and lack of organ violations.
does not pose direct threat for life. Complications meet in the absence of the adequate or out of time begun treatment more often. But even at successfully carried out therapy not achievement of positive dynamics is possible. Preservation of contractures prevents the child to carry out the simplest movements. Full immobilization of the injured joints and irreversible deformation of extremities are considered as serious complications. At a heavy current also damage of rostkovy zones of bones and secondary defeat of epifiz meet.
Artrogripoz usually comes to light at planned ultrasonography screening. On an ekhografiya at a fruit low-mobility, reduction of soft tissues of extremities in volume and development of contractures is registered. After the birth examination is conducted together with the children's neurologist, the orthopedist or the surgeon. Diagnostic criteria are existence of contractures more than three large articulate surfaces (the brush or foot are considered for 1 large joint), the defeats of motor-neurons of a spinal cord instrumentalno confirmed and an atrophy of muscles. For specification of correctness of the diagnosis the following groups of methods are used:
- Visualizing. As diagnostics of the first line the X-ray analysis of modified joints is carried out. With its help it is possible to reveal an underdevelopment of apofiz, osteoporosis and violation of processes of a differentiation of a skeleton. KT or MRT are applied in disputable cases, allow to find the slightest changes in articulate surfaces.
- Neurophysiological. For the purpose of identification of violations of neuromuscular conductivity use an elektroneyromiografiya (ENMG). It is necessary for confirmation of damage of a spinal cord. Apply an electroencephalography (EEG) to registration of activity of nervous cells of the central nervous system. Include the EEG-monitoring allowing to watch brain activity within a day in diagnostics less often.
The differential diagnosis is carried out with poliomyelitis consequences, a diastrofichesky dysplasia, Larsen's syndrome, hondrodistrofiy, spinal amyotrophy, miotonichesky dystrophy. The help of the infectiologist, cardiologist and rheumatologist can be necessary for final diagnosis.
Treatment of an artrogripoz
Medical actions begin from first days after the birth since result of correction during this period the stablest. To age of 2-3 years it is more preferable conservative techniques. They consist in carrying out the landmark plaster correction which is carried out weekly. Before each manipulation thermal, physiotherapeutic procedures and remedial gymnastics are made. From physiotreatment apply photochromotherapy, an electrophoresis with prozeriny, trentaly, sulfur and calcium, with keratolitichesky gels and electrostimulation. Without fail explain to parents about importance of carrying out corrective exercises at an artrogripoza which are carried out to 8 times a day. Orthopedic therapy is always supplemented neurologic, including the medicines strengthening blood supply, increasing conductivity and normalizing to a traffic of fabrics.
At negative dynamics apply surgical intervention. In difficult situations it is begun from 3 months, but in most cases conservatively conduct patients up to 2 years. Correction is originally carried out on disteel sites. Depending on localization performance of a miotenoligamentokapsulotomiya, nadmyshchelkovy osteotomiya, lengthening by the four-head of a muscle is possible. If there was an artrogripotichesky clubfoot, then surgeries on the copular and tendinous device (to children up to 7 years) or on a foot skeleton are recommended (in the senior age group).
At an artrogripoza of the top extremities apply operations on shortening and lengthening of muscles of a forearm, and also a kapsulotomiya of a luchezapyastny joint from the palmar party. At teenagers as operations on this joint are used by a mylar tape or . At a razgibatelny contracture of an elbow joint extend a triceps sinew, carry out its change and carry out a back kapsulotomiya. When developing dislocations their closed reposition is performed only with the safe force of muscles. The lower extremity operations are most directed on preservation of basic and static functional activity, top - on creation of conditions which are important for self-service.
Forecast and prevention
The forecast directly reflects severity of a disease. At local forms it favorable, at generalized comes the patient's invalidization. Prevention consists in the supporting mode. At a disease of the lower extremities use of orthopedic footwear, top ‒ application steak is obligatory, acquisition of special proofreaders of a bearing or corsets is important for fixing of a backbone. Apply medical massage, physical culture and gymnastics to maintenance of a satisfactory form of nervous and muscular devices.