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Disease of Madelunga


Madelunga disease (synonym: deformation of Madelunga, a chronic incomplete dislocation of a brush)  the local fizarny dysplasia which is characterized by shortening of a beam bone and dislocation (incomplete dislocation) of an ulna that is externally shown vystoyanny heads of an ulna and brush shift in the palmar party.

Symptoms of a disease of Madelunga:

Bone deformation causes a peculiar shtykoobrazny deformation of a radiocarpal joint which develops owing to premature closing of distal epiphyseal region of growth of a beam bone that leads to the progressing its shortening. More often one extremity is surprised. Sometimes the disease of Madelunga is combined with dysplastic scoliosis.

The disease of Madelunga occurs at girls by 34 times more often than at boys. Its first signs usually appear at the age of 9-12 years, i.e. during rapid growth of long tubular bones. Quite often the disease of Madelunga is unreasonably connected with the possible injuries of a joint postponed inflammatory processes. In the beginning note cosmetic defect, restriction of movements of a brush in the back and elbow parties. Deformation increases with age, pains and the feeling of fatigue at the movements caused by incongruence of joint surfaces develop.

The diagnosis is established on the basis of characteristic clinical and radiological manifestations. At X-ray inspection reveal relative shortening of a beam bone on 45 cm and a skoshennost of a joint surface of a distal epiphysis of a beam bone in the palmar and elbow party that makes an impression of an incomplete dislocation of a brush (wrist bones). The semi-lunar bone as if hangs over palmar edge of an epiphysis of a beam bone. Height of a distal epiphysis on the beam party considerably surpasses its height in the elbow party, and early closing of an epiphyseal rostkovy zone on the elbow and palmar party creates a palmar and elbow inclination of a joint surface. Note change of an arrangement of a proximal number of bones of a wrist, they form a wedge at which top the semi-lunar bone is located. The head of an ulna is in the provision of dislocation (in the distal direction) and is displaced in the back direction of rather radiocarpal joint. It is usually located at the level of the basis of metacarpal bones.

The disease of Madelunga should be differentiated with posttraumatic deformation like Madelunga (for example, as a result of an epiphysiolysis), beam talipomanus, bayonet shaped deformations after the postponed osteomyelitis, at tumors, etc. This disease reminds a mezomelichesky dysplasia, or a syndrome of Leri-Veyll which belongs to diskhondrosteoza. Bilateral defeat, small growth of the patient, a short brush are characteristic of Leri-Veyll's syndrome. Sometimes it is followed by deformations of tibial and humeral bones. Both beam bones at this disease are bent at an angle, opened in the elbow party therefore joint surfaces of a distal epiphysis of beam bones are slanted in the elbow party. The curvature is more expressed at the level of a distal third of a bone. Dislocation of heads of ulnas in the proximal direction because of relative shortening of these bones is noted. The brush is rejected in the elbow party.

Рентгенограмма кисти с деформацией Маделунга

The roentgenogram of a brush with deformation of Madelunga

Treatment of a disease of Madelunga:

Conservative treatment of a disease of Madelunga is inefficient. Operation should be carried out at the age of 1314. The main indications to it are the expressed cosmetic defect, dysfunction and joint pains. Earlier surgical intervention directed to elimination of cosmetic defect, for example a resection of a head of a beam bone, elbow in combination with a corrective osteotomy, often leads to considerable dysfunction of a radiocarpal joint. The osteotomy of the shortened beam bone at the level of its distal metaphysis and use of distraktsionno-compression devices allows to achieve at the same time lengthening of a bone due to formation of a bone regenerate and shift of a distal fragment of a beam bone in the back and beam party that provides congruence recovery, elimination of an incomplete dislocation of bones of a wrist and dislocation of a head of an ulna.

The forecast concerning functions after operational treatment favorable. At irrational treatment the osteoarthrosis of a radiocarpal joint develops and cosmetic defect remains.

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