What is Ollier's disease? Disease of the Olia: diagnosis and treatment

Do you feel that your little child is slowly growing legs or arms? Perhaps he has Ollier's disease. This disease, in which there are abnormalities of the development of the skeleton, that is, its dysplasia. Other names of the mentioned disease are multiple bone chondromatosis, unilateral chondromatosis or bone dyschondroplasia. Its main name was received on behalf of the Lyons surgeon Olie. He discovered and described the disease, thanks to the use of X-rays, in 1899.

What is Ollier's disease?

This disease refers to the anomalies of the development of the skeleton of bone, to which many diseases belong, including the well-known to many mothers of babies hip dysplasia.

The disease often manifests itself in childhood, because of what was previously thought that it is a disease of children. Now it is proved that pathology can make its debut in adults (signs can manifest even at the age of 20-40 years). This is a congenital pathology, but it appears later some time - in early childhood or in adolescence - that is, during intensive growth, when deformities of the bones and / or abnormal development of the arms and legs become evident.

With Olie's disease in the metaphyses of the long tubular bones of the limbs, cartilage outgrowths appear. The disease develops mainly in the bones, cartilages and joints of the hands and feet, in particular in the feet and hands, as well as in the pelvic bones, but it also occurs in the ribs, sternum or skull.

Sprains, or enchondromas, make the outer layer of the bone thin and brittle. In fact, these are benign tumors that can develop into malignant (eg, chondrosarcoma). Fortunately, this happens very rarely.

During puberty, growths are stabilized and replaced with bones.

So, Ollier's disease - a serious hereditary disease, ossification of the cartilaginous skeleton, in which also there is a thickening of the metaphyses (cervical tubular bones). The cause of its occurrence is still unknown. In a number of cases, it has an autosomal dominant genetic character.

Similar to dyschondroplasia, the diseases are rare Muffucci and Proteus syndromes. At the first, in addition to benign cartilaginous outgrowths 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 different parts of the body.

Who is affected by the disease

Earlier it was believed that chondromatosis of bones is rare. But now, when the X-ray study is conducted quite widely, it turned out that it can not be called a rarity. If 20 years ago, 30 cases were described, now there are twice as many.

It is usually impossible to determine the exact beginning if a regular dynamic X-ray examination has not been carried out since birth, especially in the early childhood and intrauterine development, when, in fact, Olie's disease arises. This disease, incidentally, in girls is detected almost twice as often as in boys.

Symptoms of the disease

In order not to miss symptoms of the disease, parents should be alerted if the child:

• Some limb / limb sections are delayed in development;
• There is an asymmetry and shortening in them;
• Lameness is observed;
• There is a skew of the pelvis;
• There are curvatures of the joints in the valgus or varus type.

All these factors make it possible to assume that the child has Ollier's disease.

The disease does not always manifest itself immediately at birth, but already at the age of 1-4 years you can see that the child's legs or hands are growing more slowly than expected. This is the main symptom of the disease, which can not be ignored. In the rest, the baby develops normally, despite the fact that he was diagnosed with "Olie's disease".

Dischondroplasia most often affects one limb, nevertheless, cases of defeat of both are not uncommon. If the disease touched the legs, the child will be of low growth due to their shortening. Especially strongly lag behind in bone growth, if the pathological process began very early. Deformations are painful, progress slowly. Finiteness, subject to the disease, becomes shorter, thicker, gait changes, lameness 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. Muscular atrophy with this disease is not present.

The phalanges, metatarsal and, in some cases, metacarpal bones are subject to great changes. They are shortened, expanded, clumsy and contain many cartilaginous enlightenments.

Those suffering from the disease are subject to fractures, as the bone tissue develops incorrectly due to the disease. However, fractures often grow seamlessly.

Accompanying illnesses

Dischondroplasia may be accompanied by damage to other organs, although concomitant diseases do not always develop. In particular, in young girls, along with Olie's disease, ovarian teratoma and endocrine disorders, including severe ones, including very early menstruation and premature puberty with acceleration of ossification can be observed.

However, development returns to normal with the removal of the ovarian tumor.

Diagnostics

To identify the disease of Olie, first of all you need to do an x-ray. A bone biopsy and magnetic resonance imaging should also be performed. If a disease is found, patients need regular check-ups with the doctor, in order to timely identify malignant transformations in the chondrosarcoma, if necessary.

Differential diagnostics

Differential diagnosis in the disease of Olie is, in fact, not needed after the X-ray examination, as the picture of chondromatosis is very specific. Nevertheless, each case is individual, and the set of painful symptoms is almost not repeated, so doctors should exclude a number of similar diseases.

X-ray excludes atypical chondrodystrophy, hemygypertrophy or bone hemiatrophy, rachitic deformities and some other diseases. Also, due to X-ray images, Recklinghausen's disease is excluded, which can usually be confused with the disease of Olie. The main principle is to make an x-ray of the entire skeleton, not part of it.

Principles of treatment

Dischondroplasia now, unfortunately, does not have methods of rational treatment. In contrast, for example, from such a disease as hip dysplasia, chondromatosis is treated only by surgical correction of deformation. In some cases, artificial endoprosthetics are indicated. The rest of the treatment that the patient should receive is symptomatic and supportive.

Surgical intervention is shown mainly to adults, as, according to modern observations, with stable correction in school age, stable results were not obtained, which means that the sense of performing surgical treatment is only with deformations that have formed, but not dynamic ones. However, with severe lesions, surgical intervention is also possible for children, especially when it comes to fingers that are significantly distorted in the case of Ollier's disease.

The disease requires the maintenance of joints and bones: patients are shown wearing orthopedic shoes, using an orthopedic device and other devices to fasten the joints. Patients must necessarily engage in the exercises designed specifically for this pathology of the therapeutic and sports complex.

Useful exercises and gymnastics and exercises on special simulators. This helps restore muscle tone and strengthen the ligaments.

Forecast

According to research, most often to pubertal age cartilage tissue is replaced by bone (perhaps, this is why in adults, cases of the disease are extremely rare). However, in young children, the prognosis is less encouraging: deformation, although slowly, but progresses. This is exactly what Ollier's disease is dangerous for.

The disease is so individually manifested in each case, that on the whole the forecast is unambiguously difficult to estimate. With multiple lesions, it is more favorable than in the case of localized single changes. Single deformations lead to a shortening of one of the limbs and, consequently, to their asymmetry, especially when it comes to young children. And this is impossible to correct. Also, early development of tumors increases the risk of various deformities of the fingers.

Transformation into chondrosarcoma

Despite the fact that transformation into a malignant form - chondrosarcoma - occurs infrequently, there is still a chance of cancer. Mostly this happens in adolescence, although a number of modern studies show that this can happen much later. For this reason, patients with dyskondroplasia are recommended regular consultations with a doctor and medical examinations.