Do you feel like your little baby is slowly growing legs or arms? Perhaps he has Olier's disease. This is a disease in which anomalies in the development of the bone skeleton occur, that is, its dysplasia. Other names for the disease mentioned are multiple chondromatosis of bones, unilateral chondromatosis, or dyschondroplasia of bones. The disease got its main name from the name of the Lyon surgeon Olier. He discovered and described the disease, thanks to the use of X-rays, in 1899.
What is Olier's disease
This disease refers to anomalies in the development of the bone skeleton, which includes many diseases, including hip dysplasia, well-known to many mothers of babies.
The disease often manifests itself in childhood, which is why it was previously thought that it was a disease of children. It has now been proven that pathology can also debut in adults (signs can appear even at the age of 20-40 years). This is a congenital pathology, but it is detected after some time - in early childhood or adolescence - that is, during intensive growth, whenbone deformities and/or abnormal development of arms and legs become apparent.
When Olier's disease in the metaphyses of the long tubular bones of the limbs, growths of cartilage appear. The disease develops mainly in the bones, cartilage and joints of the arms and legs, in particular in the feet and hands, as well as in the bones of the pelvis, but it also occurs in the ribs, sternum or skull.
Growths, or enchondromas, make the outer layer of bone thin and brittle. In fact, these are benign tumors that can develop into malignant ones (for example, chondrosarcomas). Fortunately, this rarely happens.
During puberty, the growths stabilize and are replaced by bones.
So, Ollie's disease is a severe hereditary disease, ossification of the cartilaginous skeleton, which also causes thickening of the metaphyses (necks of tubular bones). The reason for its occurrence is still unknown. In some cases, it has an autosomal dominant genetic character.
Similar to dyschondroplasia diseases are rare Maffucci and Proteus syndromes. In the first case, in addition to benign cartilage growths and skeletal deformities, hemangiomas are formed on the skin - dark red spots of irregular shape, consisting of blood vessels. In the second, benign tumors develop in various parts of the body.
Who is affected
It used to be that bone chondromatosis was rare. But now, when the X-ray examination is carried out quite widely, it turned out that it cannot be called a rarity. If 20 years ago 30 cases were described, now there are twice as many.
Determining the exact onset is usually impossible unless regular dynamic X-ray examinations have been performed since birth, especially in early childhood and fetal development, when Olier's disease actually occurs. This disease, by the way, is detected in girls almost twice as often as in boys.
Symptoms of disease
In order not to miss the signs of the disease, parents should be alert if the child:
- delayed in the development of some parts of the limb/limbs;
- there is asymmetry and shortening in them;
- there is lameness;
- there is a pelvic tilt;
- there are curvature of the joints of the valgus or varus type.
All these factors suggest that the child has Olier's disease.
The disease does not always appear immediately at birth, but already at the age of 1-4 years, you can notice that the child's legs or arms grow more slowly than expected. This is the main symptom of the disease, which should not be ignored. Otherwise, the baby is developing normally, despite the fact that he was diagnosed with Olier's disease.
Dyschondroplasia most often affects one limb, however, it is not uncommon for both limbs to be affected. If the disease has touched the legs, the child will be short due to their shortening. Bones are especially lagging behind in growth if the pathological process began very early. The deformities are painful and progress slowly. limb subject todisease, it becomes shorter, thicker, gait changes, lameness is observed. There may be varus or valgus deformity of the knee (less often - the proximal end of the thigh or foot), scoliosis of the spine. Muscle atrophy is not present in this disease.
The phalanges, metatarsal and, in some cases, metacarpal bones undergo great changes. They are shortened, widened, clumsy and contain a lot of cartilage enlightenment.
Suffering from Olier's disease, they are prone to fractures, because as a result of the disease, their bone tissue does not develop correctly. However, fractures most often heal without problems.
Comorbidities
Dyschondroplasia can be accompanied by lesions of other organs, although concomitant diseases do not always develop. In particular, in young girls, together with Olier's disease, ovarian teratoma and endocrine disorders, including severe ones, can be observed, including very early menstruation and premature puberty with accelerated ossification.
However, development returns to normal when the ovarian tumor is removed.
Diagnosis
To detect Olier's disease, first of all, an x-ray must be taken. Bone biopsy and magnetic resonance imaging should also be performed. If a disease is detected, patients need regular examination by a doctor in order to timely detect malignant transformations into chondrosarcoma if necessary.
Differential Diagnosis
Differential diagnosis in Olier's disease, in fact, is not needed after an X-ray examination, since the picture of chondromatosis is very specific. However, each case is different, and the set of painful symptoms is almost never repeated, so doctors should exclude a number of similar diseases.
X-ray excludes atypical chondrodystrophy, hemihypertrophy or hemiatrophy of bones, rachitic deformities and some other diseases. Also, thanks to x-rays, Recklinghausen's disease, which can usually be confused with Olier's disease, is excluded. The main principle is that it is necessary to take an x-ray of the entire skeleton, and not part of it.
Principles of treatment
Dischondroplasia currently, unfortunately, has no rational treatment. Unlike, for example, such a disease as hip dysplasia, chondromatosis is treated only with surgical correction of the deformity. In some cases, artificial endoprosthesis is indicated. The rest of the treatment that the patient should receive is symptomatic and supportive.
Surgical intervention is indicated mainly for adults, since, according to modern observations, no stable results were obtained with surgical correction at school age, which means that it makes sense to carry out surgical treatment only with formed deformities, but not dynamic ones. However, in case of severe lesions, surgery is also possible for children, especially when it comes to fingers, which are significantly curved in Olier's disease.
The disease requires the maintenance of joints and bones: patients are indicated to wear orthopedic shoes, the use of orthopedic apparatus and other devices to fix the joints. Patients must be engaged in the exercises of the therapeutic and physical culture complex designed specifically for this pathology.
Gymnastics and exercises on special simulators are also useful. This helps to restore muscle tone and strengthen ligaments.
Forecast
According to research, cartilage tissue is most often replaced by bone tissue by puberty (perhaps this is why cases of the disease are extremely rare in adults). However, in young children, the prognosis is less rosy: the deformity, although slowly, is progressing. This is what Ollie's disease is dangerous for.
The disease manifests itself so individually in each case that it is unequivocally difficult to assess the prognosis in general. With multiple lesions, it is more favorable than in the case of localized single changes. Single deformities lead to shortening of one of the limbs and, consequently, to their asymmetry, especially when it comes to small children. And this is already impossible to fix. Also, with the early development of tumors, the risk of various deformities of the fingers increases.
Transformation into chondrosarcoma
Despite the fact that transformation into a malignant form - chondrosarcoma - occurs infrequently, there is still a chance of developing cancer. It mostly occurs during adolescence, although a number ofcurrent research shows that this can happen much later. For this reason, regular consultations with a doctor and medical examinations are recommended for patients with dyschondroplasia.
