Chondromatosis bones

Chondromatosis bones (gr. chondros - cartilage; synonym: the disease of Ollie, internal chondromatosis bones, dyschondroplasia) - congenital systemic skeletal disease manifested mainly unilateral violation encontrarnos ossification, mainly in some large tubular bones of the limbs, and as a consequence - the slowdown of their growth in length.
The etiology of the disease, and the reason is mainly unilateral lesions of the skeleton is not clear.
The greatest changes are both metaphysis tibial and distal mutatis thighs. To a lesser extent, affects the proximal mutatis of the humerus and the distal ends of the bones of the forearm, and both metaphysis fibula. In the fingers of the hands and feet, and metacarpal and metatarsal bones possible changes throughout their course. In severe cases, the affected vertebrae, pelvis, shoulder, ribs, heel and ankle bones. The bones of the cranial vault and collarbone with connective tissue origin, in the pathological process is not involved.
Chondromatosis bones was first described as a one-sided defeat. Typically, however, mainly a massive defeat on the one hand, combined with less severe lesions of bones and other hand.
Pathological anatomy. When chondromatosis bones in the bones and cartilage of origin formed pockets of cartilage tissue. Pockets of chondromatosis are deep in the bones, sometimes form a protrusion on the surface, reminding bone and cartilage exostosis. The centers are located in the area of metamizol or divisov. Macroscopically they are like hyaline cartilage. The microscopic structure of these lesions are often indistinguishable from chondroma. In rare cases of chondromatosis bones may be combined with depigmentation of the skin and formation in it of the cavernous angioma. This variant was named the syndrome Maffucci.
Chondromatosis bones is evident from the moment of birth, the centers are increasing along with the growth of the skeleton at the end stops and the growth of the foci of chondromatosis. In some cases, in one of the centres of chondromatosis later than may tumor - chondroma or chondrosarcoma.
Clinical course. The main symptoms of chondromatosis bones - stunting affected limb, shortening and thickening her (compared with healthy limb), as well as the varus or deformity of the knee, and sometimes ankle, Tazo-hip joints. Changing gait, appears limp. Movements in the joints saved in full. The difference in length is healthy and diseased limbs increases with the growth of the skeleton and is stable with the termination of its development. With significantly expressed deformation of patients sometimes complain of pain, which can never be significant. Clinical manifestations of the disease are most pronounced in adults, in children as they are insignificant.
Accurate diagnosis of chondromatosis bones possible only with the help of x-ray studies. X-ray picture of the disease is quite characteristic. Affected long tubular bones shorter normally developed similar bones of the other party. Metaphysi bulavovidnyj thickened, vzduchu from the inside. DiViSy curved. In locations chondromatosis sites normal bone figure substituted longitudinally arranged oval patches of enlightenment (rarefaction). These sites neomogene, drawing them alight with bone rungs coming fan from diaphysis to metafizare germ area. Sometimes on the background of these prosvetlenie there are lime blotches. Cortical layer above chondromatosis nodes thinned and is elevated from the inside, which causes the thickening of the metaphysis. Periosteal reaction never happens. In the iliac bone and shoulder blades areas of depression are located closer to the periphery, in a Central departments is maintained normal bone pattern. Ribs are affected at the front end, which, as a rule, are sharply swollen.
The prognosis for chondromatosis bones favorable. In rare cases it is possible rebirth separate chondromatosis nodes in the true cartilaginous tumors that may become malignant and move in chondrosarcoma (see).
Signs of malignization - establishment of permanent gradually increasing pain and swelling in one of the bones. On radiographs among old changes in the damaged bone identify areas of destruction, which quickly grow in size with the destruction of the cortical layer.
Treatment. In malignant growth and with considerable obezvrezhivaniju surgical treatment.
In practice doctor chondromatosis bones is rare. More often it is necessary to observe chondroma (see).