Congenital kosorukost – steady deformation as a result of which the brush deviates in the elbow or beam party a longitudinal axis of a forearm. Arises in the pre-natal period. Is a consequence of an underdevelopment of one of bones of a forearm or the sinews associated with these bones. It is shown by a curvature of the top extremity – the brush is located at an angle to a forearm. It can be combined with an underdevelopment of pyastny bones, phalanxes of fingers, lack of one or several fingers, an union of fingers, contractures, incomplete dislocations or dislocations in elbow and luchezapyastny joints. The diagnosis is exposed on the basis of a radiological picture and data of an objective research. Treatment is surgical, it is carried out at early age (usually till 1 year). After operation LFK, massage and physical therapy is appointed.
Congenital kosorukost – anomaly at which the brush settles down at an angle to a forearm. This type of deformation of extremities. Arises owing to an underdevelopment of bones and/or sinews of a forearm in the pre-natal period. Is rare pathology, according to literary data the beam kosorukost comes to light at one of 55 thousand newborns, an elbow kosorukost – at one of 220-550 thousand newborns. Can be unilateral or bilateral. It is quite often combined with other congenital malformations. Treatment is performed by children's orthopedists. Surgery at early age is recommended.
The congenital kosorukost is formed as a result of influence of a number of external and internal factors. Insufficient food of mother, infectious diseases, reception of some medicines, and also the ionizing radiation belongs to number of external (exogenous) factors which can cause development of this pathology. Among the internal (endogenous) reasons – late pregnancy, hormonal violations, a serious somatic illness of mother, functional disorders and pathological changes of a uterus. Influence time is of great importance – the first 5 weeks of pregnancy are considered as the critical period. Genetic predisposition is not revealed.
Allocate two types of a congenital kosorukost: beam and elbow. The beam kosorukost is formed at an underdevelopment or lack of a beam bone and the sinews associated with it, elbow – at an underdevelopment or lack of an elbow bone and the corresponding sinews. Each type of a congenital kosorukost is subdivided into several subtypes taking into account bone underdevelopment degree.
- Moderate hypoplasia. The elbow bone is shortened for 10-29% in comparison with beam.
- The expressed hypoplasia – an elbow bone is shortened for 30-69% in comparison with beam.
- Rudiment of an elbow bone – the elbow bone is shortened for 70-99% in comparison with beam.
- Aplaziya of an elbow bone – an elbow bone completely is absent.
- 1 degree – a beam bone is shortened no more, than for 50% of norm.
- 2 degree – a beam bone are shortened more, than for 50% of norm.
- 3 degree – a beam bone completely are absent.
Besides, at a beam kosorukost experts in the field of orthopedics and traumatology allocate 4 types of a brush:
- 1 type – a hypoplasia of the first pyastny bone and the tenor's muscles (the eminence located between the I finger and a luchezapyastny joint).
- 2 type – the I pyastny bone are absent, the underdevelopment of phalanxes of 1 finger comes to light.
- 3 type – the I pyastny bone and the I finger are absent, absence II and III fingers can be also observed.
- 4 type – bones of a brush are developed normally.
At a beam kosorukost the brush is in position of the pro-nation, between a brush and a forearm the corner opened towards a beam surface (side of a thumb) is formed. On a back surface the proximal shift of a brush in relation to a head of an elbow bone comes to light. The brush rotates around a head as a weather vane tag. At most of patients with a congenital kosorukost various defects of a brush are observed: lack of the I finger, lack of the I pyastny bone, absence I, II and III fingers. The I finger can be shortened, consist of one phalanx or due to the lack of the I pyastny bone to hang on a skin leg. V and IV fingers at the same time are developed normally. Because of an underdevelopment and contractures of fingers functionality of a brush is sharply limited, capture of objects is often impossible.
The forearm is usually shortened, the head of an elbow bone acts. Because of deformation of an elbow bone there is the arc-shaped curvature of a forearm with the camber turned into the elbow party (side of the V finger). Muscles of a forearm are underdeveloped. Pronatora and forearm instep supports, long muscles of the I finger and a plecheluchevy muscle quite often are absent. Perhaps also lack of a long head of a biceps of a shoulder. At an aplaziya of a beam bone lack of a beam artery is also observed.
At an elbow kosorukost the brush is in the provision of a supination, between a brush and a forearm the corner opened in the elbow party (side of the V finger) is formed. V and IV fingers often are absent or are underdeveloped. The forearm is shortened. Because of deformation of a beam bone the arc-shaped curvature turned by camber into the beam party (side of the I finger) is formed. The movements in an elbow joint are limited because of dislocation of a head of a beam bone. Forearm muscles from the elbow party are underdeveloped or are absent. Function of a brush is broken less, than at a beam kosorukost.
Diagnosis a congenital kosorukost does not represent difficulties because of visually visible deformations and obvious dysfunction of an extremity. For exact assessment of degree of an underdevelopment of bone structures and specification of the further plan of treatment carry out a X-ray analysis of bones of a forearm and a X-ray analysis of bones of a brush. For assessment of a condition of soft fabrics appoint MRT of a forearm and an electromyography.
treatment should be carried out only under the supervision of a doctor. Self-treatment is unacceptable!!!
Treatment of a congenital kosorukost begins from the first days of life. To the babies who did not reach 6-month age the conservative therapy directed to "extension" of soft fabrics, reduction and prevention of development of contractures is appointed. Patients go to LFK, massage and carrying orthoses. The optimum period – is recommended to perform 6-9 months operations aged till 1 year. The indication to surgical intervention are nonremovable contractures in luchezapyastny and elbow joints, a nekorrigiruyemy deviation of a brush in relation to a forearm and restrictions of function of a brush. Depending on degree and a type of deformation intervention can be both one-stage, and multi-stage. At the first stage liquidate the most functionally significant deformation, at the subsequent stages carry out correction of small deformations and, at an opportunity – cosmetic defects.
At a beam kosorukost the surgical interventions directed to restoration of "fork" in a luchezapyastny joint are carried out. At the expressed contractures intervention on bones is carried out in combination with lengthening of a sgibately brush and skin plasticity. At an aplaziya of a beam bone the radialization or centralization of a brush is carried out. Within 1-2 months the brush is removed in the correct situation, using the distraktsionny device (Ilizarov's device), and then stabilize it on an elbow bone. The expressed arc-shaped curvature of a forearm is the indication for carrying out a corrective osteotomiya.
At an elbow kosorukost excision of the fibrous tyazh connecting an underdeveloped elbow bone to a luchezapyastny joint in combination with a corrective osteotomiya of a beam bone is carried out. If length of an elbow bone makes more than 50% of norm, the bone is extended, using Ilizarov's device. Sometimes intervention should be carried out in two steps: at the first stage the bone is removed in the correct situation and eliminate beam head dislocation, on the second form a one-bone forearm.
Sindaktiliya (union of fingers) at an elbow and beam kosorukost usually eliminate at the final stage, after correction of more significant deformations. To the place of the absent fingers replace fingers from foot. In the postoperative period surely appoint physical therapy, massage and LFK for prevention of contractures and secondary deformations.