2Fibrous bone dysplasia
- 2.1Classification of fibrous bone dysplasia
- 2.4Treatment of bone dysplasia
- 2.5Which doctor heals
3Fibrous dysplasia: symptoms and treatment
- 3.2Classification in children and adults
- 3.4Diagnosis based on the presence of symptoms
- 3.5Treatment with conservative methods
- 3.6Prophylaxis and prognosis
4Fibrous bone dysplasia (osteodysplasia)
- 4.1The history of the discovery of pathology
- 4.2Nosological classification of pathological changes
- 4.3Symptoms of fibrous dysplasia of the femoral and tibia in children
- 4.4Fibrous dysplasia of the bones of the skull, upper and lower jaws
- 4.5Treatment of fibrous bone dysplasia
Fibrous dysplasia- damage to the bone, in which a portion of normal bone tissue is replaced by a connective tissue with the inclusion of bone trabeculae.
Fibrous dysplasia belongs to the category of tumor-like diseases, can be local or common, affecting one or more bones. The causes of development are not clear, genetic predisposition is not ruled out.
Is manifested by pain, deformity, shortening or elongation of the segment and pathological fractures. The diagnosis is made on the basis of radiography, MRI, CT and other studies. Treatment is usually surgical.
Fibrous dysplasia is a systemic lesion of the skeleton, which belongs to the category of tumor-like diseases, but is not a true tumor of the bones. It arises from the incorrect development of the osteogenic mesenchyme (tissue, from which the bone subsequently forms).
Symptoms usually come to light early in childhood, but there may be a late onset. In the literature, cases are described where mono-osseous fibrotic dysplasia was first diagnosed in people of retirement age. Women are sick more often than men.
It is possible to degenerate into a benign tumor; Malignancy is rare.
The disease was first described in the first half of the 20th century. In 1927, the Russian surgeon Braitsov made a report on the clinical, microscopic and roentgenologic signs of focal fibrotic bone degeneration.
In 1937, Albright described multifocal fibrous dysplasia, combined with endocrine disorders and characteristic skin changes.
In the same year, Albrecht described multifocal dysplasia in combination with premature puberty and fuzzy skin pigmentation.
A little later, Jaffe and Liechtenstein investigated single-frontal lesions and published conclusions about the causes of their occurrence. In the literature, fibrous dysplasia may occur under the names Liechtenstein's disease, Liechtenstein-Jaffe disease or Liechtenstein-Braitsov disease.
There are two main forms of fibrous dysplasia: mono-osseous (with damage to one bone) and polyosseous (with the defeat of several bones, usually located on one side of the body).
The polyosseous form develops in childhood and can be combined with endocrine disorders and melanosis of the skin (Albright's syndrome).
The mono-osseous form can manifest at any age, endocrinopathies and skin pigmentation are not observed in patients.
Russian specialists use the clinical classification of Zatsepin, which includes the following forms of the disease:
- Intraosseous form. Can be mono-osseous or polyosal. Single or multiple foci of fibrous tissue are formed in the bone, in a number of cases the degeneration of the bone all over, but the structure of the cortical layer is preserved, so the deformations are absent.
- Total bone damage. Suffer all the elements, including the cortex and the area of the medullary canal. Because of total defeat, deformations are gradually formed, often fatigue fractures occur. Typically, a polyosseous lesion of long tubular bones is observed.
- Tumor form. It is accompanied by proliferation of foci of fibrous tissue, which sometimes reach considerable dimensions. It is rarely found.
- Albright Syndrome. It manifests itself as a polyosseous or almost generalized bone lesion in combination with endocrine disorders, premature sexual maturation in girls, a violation of body proportions, focal pigmentation of the skin, severe deformities of the bones of the trunk and extremities. It is accompanied by progressive disorders from various organs and systems.
- Fibrous-cartilaginous dysplasia. Appears the primary degeneration of cartilage, often there is a degeneration into the chondrosarcoma.
- Calcifying fibroids. A special form of fibrous dysplasia is very rare, usually affects the tibia.
Expressed congenital deformations are usually absent. With the polyossal form, the first symptoms appear in childhood. The defeat of the bones is accompanied by endocrinopathies, skin pigmentation and disruption of the cardiovascular system.
Manifestations of the disease are quite diverse, the most constant signs are minor pain (usually in the hips) and progressive deformities. Sometimes the disease is diagnosed only if a pathological fracture occurs.
Usually, in polyosseous form, tubular bones are lesioned: tibial, femoral, peroneal, brachial, radial and ulnar. Of the flat bones, pelvic bones, skull bones, spine, ribs and scapula often suffer.
Often, the damage to the bones of the hands and feet is detected, while the wrist bones remain intact. Degree of deformation depends on the location of foci of fibrous dysplasia. When a process occurs in the tubular bones of the upper limbs, only their clavate extension is usually observed.
When the phalange is affected, the fingers are shortened, they look "chopped off".
The bones of the lower limbs are curved under the weight of the body, characteristic deformations occur. Especially sharply deforms the femur, in half of cases its shortening is revealed.
Due to the progressive curvature of the proximal parts, the bone takes the form of a boomerang (shepherd's staff, hockey stick), a large spit "moves" up, sometimes reaching the level pelvic bones.
The neck of the thigh is deformed, there is lameness. Shortening of the hip can be from 1 to 10 cm.
In the formation of the focus in the fibula, there is no deformity of the limb, with damage to the tibia can be observed saber-like curvature of the tibia or slowing of bone growth in length.
Shortening is usually less pronounced than with a focus in the femur. Fibrous dysplasia of the ileum and ischium bones causes deformation of the pelvic ring.
This, in turn, negatively affects the spine, triggering disorders of posture, scoliosis or kyphosis.
The situation is aggravated if the process simultaneously affects the hip and pelvic bones, since in such cases the body axis is even more disturbed, and the load on the spine increases.
The mono-osseous form proceeds more favorably, there are no extraarticular pathological manifestations.
The severity and nature of deformations vary greatly depending on the location, the size of the focus and the characteristics of the lesion (total or intraosseous).
There may be pain, lameness and fatigue after the load of the affected segment. As with the poliossal form, pathological fractures are possible.
The diagnosis is made on the basis of the clinical picture and the data of the X-ray study.
At the initial stage, on the X-ray images in the area of the diaphysis or metaphysis of the affected bone, zones that resemble frosted glass are identified.
Then the affected area acquires a characteristic speckled appearance: the foci of compaction alternate with the areas of enlightenment. Deformation is clearly visible.
When a single focus is detected, multiple bone lesions must be excluded, which can be asymptomatic in the initial stages, so patients are referred for densitometry. If there are suspicious areas, X-rays are performed, if necessary, CT scans are used.
It should be borne in mind that fibrotic dysplasia, especially mono-osseous, can present significant difficulties in the diagnosis process.
With a mildly expressed clinical symptomatology, a prolonged dynamic observation is often required.
To exclude other diseases and assess the condition of various organs and systems, consultations with a phthisiatrist, oncologist, therapist, cardiologist, endocrinologist and other specialists may be required.
Treatment is predominantly surgical - complete resection of the affected area of the bone within healthy tissue and replacement of the defect with an osteal graft. In the pathological fracture impose an apparatus Ilizarov.
With multiple lesions, preventive measures are taken to prevent deformities and pathological fractures. The prognosis for life is favorable.
In the absence of treatment, especially in the polyosal form, gross, crippling deformations can occur. Sometimes foci of dysplasia degenerate into benign tumors (giant cell tumor or non-fissile fibroma).
In adults, several cases of malignant degeneration into osteogenic sarcoma have been described.
A source: http://www.krasotaimedicina.ru/diseases/traumatology/fibrous-dysplasia
Fibrous bone dysplasia
For the first time, fibrotic bone dysplasia was reported in 1927 at the 19th Congress of Russian Surgeons. This report was owned by VR Braitsev, whose name is now called. But it was not always so.
Despite the fact that fibrous bone dysplasia was first described by Braits, for some time it was called Liechtenstein-Jaffe disease. These two scientists only supplemented the description given by Braits. In the subsequent on the initiative of TP.
Vinogradova in 1973, this pathology was renamed into the disease of Braitsev-Lichtenstein in the whole world literature.
According to the discoverer, the cause of the development of osteophysical dysplasia was considered the disturbed work of the mesenchyme, which reproduces the fibrous-type bone tissue. As a result, the latter has a complete and incomplete structure.
Therefore, fibrosing of the bone marrow occurs with a tendency to expand and form a typical osteoid. Similar disorders develop in the embryonic period under the influence of uncertain factors.
They can spread either to the bone site, or to the entire bone, or to involve several bones in a row.
Classification of fibrous bone dysplasia
To date, there is no single classification of fibrous bone dysplasia, which would satisfy all the requirements of clinicians. Different variants of the division of this pathology are suggested. The most common are the following.
Depending on the number of bones involved, the disease is divided into the following types:
- Monoostic - the process affects only one bone
- Axial - long bones of the tubular structure of one of the extremities are affected, for example, arms or legs
- One-sided - bones from one side are affected, for example, bones of the right upper and lower limbs
- Two-sided - the bones of the right and left side are affected.
He does not distinguish between the following forms:
- Albright syndrome - characteristic lesions of the bone, in which there is increased skin pigmentation and pubertal period in girls, which begins before the age norm
- Polyossal - only bones are involved in the process, with 2 or more
- Monoossal - only one bone is affected.
In modern medicine, the most rational is considered to be a classification based on clinical and roentgenological data. According to her, the following forms are distinguished:
- Monoossal - the disease involves only one bone
- Oligossal - 2 or 3 bones
- Polyosal, within the framework of which forms are isolated without extra-ocular manifestations and forms with such, for example, incomplete variant of Albright syndrome
- Albright Syndrome
- Mixed form, in which not only bone tissue is involved in the pathological process, but also cartilaginous (bone-cartilaginous dysplasia).
The course of the disease in different cases can be different. For some unknown reason, some patients experience a slow course, while others have a rapid progression of the pathological process.
Such rapid growth is combined with a pronounced cellular polymorphism, which brings it closer to the oncological process (with sarcoma of the bone). Most often, the pathological process involves the tibia, which leads to a characteristic appearance of the patient.
However, the nature of the clinical picture depends on the localization of the lesion. Common clinical symptoms are:
- Changing the shape of bones
- Lameness (if the process affects the bones of the lower limbs, which leads to their shortening or elongation)
- Pain in the place of injury
- Pathological fractures.
Pain syndrome in fibrous dysplasia of bones is characterized by certain signs. These are the following:
- The most common characteristic of the defeat of the bones of the lower extremities and the skull is practically absent if the upper extremities or bones of the trunk are involved in the pathological process
- Pain has a different duration and, accordingly, the degree of patient restriction is also significantly different
- In the absence of physical activity and during rest, the pain does not bother the person
- Pain is intensified if a person commits any movement or lifts the gravity
- The presence of lameness and deformation contributes to the intensification of pain.
The defeat of the tibia, which is a favorite localization of fibrous osteodysplasia, leads to its curvature anteriorly, as well as to the side.
There is a lateral flattening of the bone and areas of uneven expansion.
Pain can occur either simultaneously with the appearance of deformation, or after some time before or after its appearance.
The change in the shape of the femur leads also to a characteristic deformation. It consists in a pronounced curvature of the thigh in the outer side.
In this case, a large spit can reach the almost iliac wing. This leads to a significant change in gait.
Such deformation in the medical literature has different names, for example, the shape of a hockey stick, a jug handle, etc.
The pathological process affecting the humerus causes it to expand like a mace. At the same time, the structure of the brush is also broken, the appearance of which resembles the chopped off fingers. This is due to the shortening and thickening of finger phalanges.
Bony curvatures become the cause of loose joints.
This creates conditions for the development of the degenerative-dystrophic process in them (deforming osteoarthritis), which further disturbs the human condition.
The situation can be aggravated with incorrectly fused fractures, in which the bone is finally curved and shortened.
With fibrous dysplasia of bones, more rare forms of the disease can be noted, in which the following anatomical structures are affected:
- Bone pelvis.
It is important to note one feature of fibrous osteodysplasia. This disease always begins from childhood, then its progression is observed (slow or rapid depending on the effect of causative factors).
At the onset of puberty, the pathological process is stabilized. Therefore, the treatment of this disease should begin as early as possible in order to keep more bones as normal as possible.
Diagnosis of fibrous bone dysplasia is largely based on the results of X-ray examination.
Its carrying out in conjunction with the assessment of clinical manifestations sharply reduces the percentage of diagnostic errors.
The main distinguishing features of this disease, which can be identified on the roentgenogram, are the following:
- Varus deviation of the thigh, that is, more towards the outer surface
- Large spit is located above the normal level, approaching the wing of the ilium
- The enlargement of the osseous tubercles in the skull - frontal, occipital and parietal
- Increasing the thickness of the temporal arch
- Smoothening of the back of the nose
- Shortening of upper and / or lower limb bones
- Spotted inclusions in the general background of the bone
- A characteristic feature - "frosted glass which is determined in the figure of the affected bone
- Virtually no bone marrow canal, which is associated with abnormal bone pathological growth
- Alternation of foci of enlightenment with foci of compaction.
The presence of certain radiographic signs of the disease depends on the duration of the disease and the number of bones involved in the pathological process. It is not necessarily a combination of all the symptoms given in one patient.
Diagnosis of fibrotic osteodysplasia should be timely, as well as the subsequent treatment, since this disease belongs to the pre-tumor processes. With oncological conditions, the following symptoms bring him closer:
- The possibility of the transition of fibrotic osteodysplasia to a true tumor, especially in childhood
- Relapse of the pathological process after surgical removal of the lesion focus
- Some features of morphology
- Malignancy, that is malignancy of the process.
Thus, the illness of Braitsev-Liechtenstein is a dysplastic process in which there is real possibility of fully undeveloped bone tissue into the tumor tissue with pathologically rapid growth and division.
Treatment of bone dysplasia
Treatment of fibrous dysplasia of bones is associated with various difficulties. The largest of them is that there are no means of pathogenetic therapy, since the disease is associated with a violation of embryonic development.
In addition, the causative factors of this pathology are not completely known. So, Schlumberger believed that the basis of fibrotic osteodiplasia is the perverted reactions of bone tissue to traumatic damage.
Not all patients have normal osteoblasts, in order to fully compensate for the defect in the bone. Someone develops immature tissue, which is associated with the development of the disease.
Some authors have tried to explain the pathological changes that occur as a consequence of the malfunctioning of the endocrine system. In confirmation, they brought Albright syndrome. However, endocrine disorders were not found in all patients.
Recklinghausen considered this pathology as a local manifestation of neurofibromatosis.
At the present time, some conditional concept has been adopted regarding the causes and mechanisms of the development of this disease.
It is believed that this embryonic abnormality, which is realized after birth due to the presence of an innate neurological disorder. This is confirmed by the presence of central disorders in the pituitary and adrenal cortex.
This circumstance can open new possibilities of treatment (use of some pharmacological preparations with endocrine activity).
the task in the treatment of such patients is to reduce the severity of manifestations of this pathology and to improve the possibility of self-service and the performance of professional activities.
To achieve this, orthopedic treatment is used in most cases. Also very important are measures to prevent pathologically emerging fractures.
In this case, the tactics will be determined by the mineral density of the bone, the level of calcium and phosphorus in the blood.
Surgical methods of treatment of fibrous bone dysplasia are not routine. For their use, there are certain indications when the probability of obtaining the most favorable result is high. The main indications are:
- Rapid increase in pathological focus
- Persistent pain syndrome associated with fibrous osteodysplasia
- The presence of a fracture of a specific localization, in which there is a huge threat to the patient's life
- Presence of focal cysts.
The main stages of surgical treatment are the following:
- Removal of a portion of bone affected by the pathological process
- Deleting content
- Filling the formed cavity with a homograft
- Long period of immobilization of the limb for the formation of a strong callus.
In childhood, most often used radical surgery. It aims to completely limit the spread of the disease.
Further bone growth is provided by stimulation of osteoblasts. If false joints are formed regardless of the age of the patient, Iliazarov's apparatus is used.
They help prevent shortening of the limb.
Which doctor heals
The treatment of fibrous bone dysplasia is performed by a surgeon or traumatologist, depending on the level of specialization of the medical institution.
A source: http://www.knigamedika.ru/kost/osteo/fibroznaya-displaziya-kosti.html
Fibrous dysplasia: symptoms and treatment
- Pain in the knee
- Violation of posture
- Delayed sexual development
- Disproportionate physique
- Crunch in the affected joint
- Deformation of the structure of the face
- Disturbance of limb mobility
- Curvature of the knee
- Pathological fractures
- Impaired tooth formation
- Thickening of the jaw
- Femoral shortening
- Fractures of the tibia
- Deformation of the first vertebra
- Decrease of the cranial cavity
- Changes in the shape of the lower limbs
- Change in the volume of the paranasal sinuses
- Hanging of the cerebral part of the skull over the facial
- Increased knee mobility
Fibrous dysplasia is a malaise, characterized by a systemic lesion of the skeleton, in which a healthy connective bone tissue replaces.
The main factor in the development of the disease is a gene mutation, but clinicians identify many other sources of the disease, which may differ depending on the form of its course.
The disease is characterized by a pronounced clinical picture, in which, in addition to external deformations, pain syndrome and pathological fractures prevail.
To make the correct diagnosis is possible on the basis of symptoms, but the main role is played by instrumental examinations, in particular, MRI. Treatment in all cases involves surgical intervention.
Today, the main causes and mechanism of the onset of the disease remain unclear until the end. However, clinicians believe that the source lies in gene mutations and abnormalities of intrauterine development.
Clinicians believe that the following predisposing factors are not the last place in the formation of an ailment:
- violation of the hormonal background;
- significant violations of the formation and development of cartilage and bones;
- pathology of muscles and ligaments, articulating joints;
- genetic predisposition;
- irrational nutrition of the future mother - here it is necessary to attribute the absence in the diet of fresh vegetables and fruits, as well as other products enriched with vitamins and nutritional elements;
- addiction to bad habits and unfavorable environmental conditions affecting the female body during pregnancy;
- exacerbation of chronic ailments or suffered severe infectious diseases in the period of bearing of the child;
- uncontrolled intake of certain medications during pregnancy;
- the effect of toxic or chemical substances on the woman's body in the situation;
- The presence in the future mother of gynecological problems that are directly related to the uterus;
- severe toxicosis;
It follows that the main risk group is children, but there are cases when such a disease was first diagnosed in a person of advanced age. It is remarkable that in female representatives such pathology is diagnosed several times more often than in the strong half of mankind.
Classification in children and adults
In children and adults, several basic forms of fibrous dysplasia are distinguished:
- monoossal- characterized by the defeat of only one bone, and the symptomatology can manifest at any age;
- polioseal- differs in that several bones are involved in the pathology, for example, the hip or shoulder, but only on one side of the body.
To date, the classification of Zatsepin is widely used, which assumes his division of the ailment into:
- intraosseous dysplasia- can occur both in mono-osseous and in polyosal form. During the microscopy, there are focal lesions, but in some cases fibrous tissue can affect the whole body of the bone. Nevertheless, in patients with this type of disease, there is no expression of bone deformities;
- total damage- from the previous type differs in that all layers of the bone are affected, which causes the development of bone deformities, and this, in turn, leads to frequent fractures. Based on the name, it becomes clear that this type of disease occurs in a polyosseous form, and most often affects the tibia and femur;
- tumor form- in all cases leads to the growth of fibrous tissue. This kind of disease is extremely rare;
- Albright SyndromeIs the most common form that is diagnosed among children. Pathology is rapidly progressing and is fast-paced;
- fibrous-cartilaginous type- differs from other types of malady in that it practically in all cases passes into a malignant form;
- calcifying form- this particular form of the disease is rarely diagnosed.
Fibrous dysplasia and its manifestation in the oral cavity
The localization of the disease may also differ, most often in pathology are involved:
- large and small tibia;
- ribs and spine;
- jaw bone and skull;
- knee joint and humerus.
Fibrous dysplasia of the bones of the skull has its own classification and it happens:
- sclerotic- characterized by the formation of areas of pronounced reconstruction of bone tissue, which becomes the cause of its compaction. Most often such changes are observed at the base of the skull, nose bones, frontal and maxillary bones;
- cyst- often affects the mandibular bone. Cystiform neoplasms can be multiple and single
- paget-like- deformation of the skull occurs with Paget's disease and is expressed in a significant increase in the brain area of the skull.
Clinical manifestations of the disease will be slightly different, depending on the location of the pathological process.
Fibrous dysplasia of the tibia has the following clinical manifestations:
- shortening of the femur;
- pathological fractures;
- tibial fractures;
- lameness during walking.
Fibrous dysplasia of the knee joint is expressed in:
- painful sensations, the intensity of which increases under the influence of heavy physical exertion or with a sudden change in weather conditions;
- characteristic crunch of joints, during flexion and extensor movements of the knee;
- change the shape of not only the joint, but also the patella;
- partial or complete change in the shape of the lower limbs;
- increased mobility of the knees.
Fibrous dysplasia of the jaws is characterized by:
- a thickening of the lower or upper jaw;
- deformation of the face;
- slow process of puberty.
Fibrous dysplasia of the skull bone leads to the appearance of such signs:
- defects in the formation of teeth;
- reduction of the cranial cavity;
- overhanging the cerebral part of the skull over the facial;
- change in the volume of the paranasal sinuses;
- deformation of the first vertebra.
Often, such a variety leads to dysplasia of the hip joint and spine, which leads to various violations of posture.
In cases of localization of pathology in the humerus, the disturbance of the mobility of the affected upper limb comes to the fore.
Albright syndrome has the most pronounced symptomatology, among which:
- disruption of the endocrine system;
- premature puberty in girls;
- violation of the proportions of the trunk;
- pigmentation of cutaneous integument;
- severe deformities of bones.
In addition, this form of such a disease is often accompanied by improper operation of internal organs and systems.
Symptoms of fibrous dysplasia
Diagnosis based on the presence of symptoms
An experienced doctor will be able to correctly diagnose on the basis of the presence of specific signs. The primary examination should include:
- detailed questioning of the patient, necessary for clarifying the first time of appearance and intensity of the symptomatology;
- study of medical history and history of life - to find a predisposing etiologic factor;
- a thorough physical examination of the affected area, her palpation and percussion.
Diagnosis of fibrous bone dysplasia does not involve laboratory tests of blood, urine and feces, since they do not have diagnostic value.
Among the instrumental diagnostic techniques is:
- CT and MRI.
Treatment with conservative methods
The use of conservative methods of therapy in most cases does not give positive results, why after the establishment of the final diagnosis for an adult or child an operation is prescribed.
Treatment of fibrous dysplasia is carried out:
- resection of the affected area of the bone, followed by replacement with a bone graft;
- lengthening of the bone - only in cases of shortening.
Polyostic fibrosis dysplasia may limit the possibility of conducting operative therapy. In such cases, the treatment will include:
- wearing orthopedic shoes and other devices that reduce the burden on bones and joints;
- the course of a therapeutic massage;
- physiotherapeutic procedures;
- Exercise therapy.
Prophylaxis and prognosis
Since the mechanism of development of pathology is unknown, in order to avoid the development of such an ailment, it is necessary to follow the general rules:
- to lead a healthy lifestyle during pregnancy and not to miss visits to the obstetrician-gynecologist;
- to observe safety rules when working with dangerous substances;
- to eat properly and balanced;
- maintain normal body weight;
- To exclude the influence of excessive physical exertion on the most common places of pathology localization;
- regularly undergo a full medical preventive examination.
The outcome of fibrous dysplasia is often favorable, only the polyossal form can lead to gross crippling deformations. Complication in the form of formation of a benign neoplasm is observed in 4% of all cases, and malignancy - in,%.
Osteoarthritis of the hip joint is a disease that also occurs under the name of coxarthrosis, and usually affects people after forty years. Its cause is a decrease in the volume of allocation of synovial fluid in the joint.
According to medical statistics, women tend to suffer from coxarthrosis more often than men. It affects one or two hip joints. This disease disrupts the nutrition of the cartilaginous tissue, which causes its subsequent destruction and limits the mobility of the joint.
The main sign of ailment is pain in the groin.
A source: http://SimptoMer.ru/bolezni/sustavy-kosti/2422-fibroznaya-displaziya-simptomy
Fibrous bone dysplasia (osteodysplasia)
Fibrous bone dysplasia in children and adults can develop for many reasons.
In this case, a pathogenic lesion of bone tissue occurs, with the formation of fibrous inclusions consisting of connective tissue fibers.
This tissue, of which mainly consists of scars, remaining after wounds, cuts, burns. Substituted bone trabeculae lose strength and become a "target" for fractures.
Currently, fibrotic dysplasia is characterized by oncologists as a tumor process, although this is not entirely true.
Osteodysplasia of this type can rather be attributed to substitution processes.
But to exclude their role in the formation of the prerequisites for the emergence of osteosarcomas, too, can not be ruled out.
Characteristic symptoms depend on the form of the lesion. Localization can be single or diffuse (multiple and common).
Capture one or more bones.
Modern diagnostics with the help of roentgenography and computer tomogram allows you to detect timely all focal changes and begin the process of treatment.
Currently, doctors do not know the reasons for the beginning of pathological replacement of bone trabeculae with fibrous fibers. Therefore, no active prevention measures have been developed. But to exclude risk factors in children and adults still need.
The history of the discovery of pathology
The history of the appearance of this disease is still unknown.
The right of the first discovery of pathology is attributed to the Russian surgeon Braitsov, who at the beginning of the last century described in detail the pathological foci of bone tissue that he discovered.
He called it focal bone degeneration into fibrous tissue. In 1927, several clinical studies, including microscopic ones, were carried out.
As a result, all typical signs of fibrous osteodysplasia were classified. The disease was attributed to benign tumor processes. Accordingly, surgical procedures for its treatment were recommended.
Ten years later, in 1937, surgeons Albrecht and Albright examined in detail the multifocal forms of fibrous osteopathy in children and elderly patients. For the first time, a theory was put forward about the genetic nature of the origin of these changes.
Further research was carried out by medical scientists Liechtenstein and Jaffe, as a result of which the primary name for the identified clinical simtomokompleksu was given in the form of Liechtenstein-Jaffe syndrome. Later, it was renamed into Liechtenstein-Braitsov's disease. Currently, doctors diagnose fibrotic osteodysplasia or fibroosteodysplasia.
Nosological classification of pathological changes
By definition, fibrotic dysplasia is a systemic disease leading to the appearance of characteristic pathological changes in the bony parts of the musculoskeletal system. Bone lesion is a tumor-like process. Nosological classification allows us to subdivide them according to localization, the degree of damage to trabeculae, complications, etc.
fibrotic dysplasia of the bones of the skull and jaws
As a probable cause is the development of bone tissue at the stage of intrauterine development.
Damaged osteogenic mesenchyme, which is subsequently responsible for the growth of bone structures. Therefore, primary signs can manifest themselves in early childhood.
There are also cases when the first signs are manifested in old age. In elderly patients only mono-osseous foci of fibrotic substitution are defined.
Therefore, there is a theory and a degenerative process of bone tissue as a result of gerontological changes in the human body.
When diagnosed, doctors adhere to a certain classification. Primarily, it is conducted to determine the number of detected foci.
The congenital form is often polyosal (multiple), and acquired in the elderly - mono-osseous (single).
With a multiple focal shape, there is always a clinical picture of endocrine pathologies and melanosis of the skin. With multi-focal form, this is not observed.
Symptoms of fibrous dysplasia of the femoral and tibia in children
Often, fibrotic dysplasia in children is diagnosed at an early age. Symptoms of pathology may include a number of characteristic features that allow an experienced physician to suspect these changes.
Sometimes the pathology of the cardiovascular system comes to the forefront: the child complains of fatigue, irregular heartbeat, rapid pulse, etc.
There may be signs of hormonal dysfunction: dizziness, uneven growth, tachycardia or bradycardia, excessive body weight or weight deficit.
The long tubular bones of the lower extremities are often affected.
There fibrotic dysplasia of the femur without pronounced deformation, but with involvement in the pathological process of the hip joint.
But fibrous dysplasia of the tibia usually leads to a characteristic curvature under the weight of the body.
To defeat the tubular bones of the upper limbs, the intactness of the bones of the wrist is characteristic (the absence of their involvement in the pathological process of replacing the trabeculae with fibrous fibers).
Fibrous dysplasia of the tibia and femur. Fibrous dysplasia of pelvic bones
Characteristic symptoms are seen in the form of thickening of the bones. This is especially evident in the area of distal phalanges of the fingers - they become shortened and thickened. Larger bones become clavate.
Subjective characteristic symptoms of fibrous bone dysplasia:
- pain in the muscles of the lower and upper extremities after considerable physical exertion;
- cramps in the muscles in the evening after retirement;
- change in the color of the skin over the affected areas in the form of pigmentation;
- lameness on the background of deformation of the head of the tibia.
Objectively, shortening of the upper and lower extremities is seen. The shortening can reach 10 cm. In the future, marked scoliosis, kyphosis and lordosis are formed. The flat pelvic bones and symphysis are affected.
Fibrous dysplasia of the bones of the skull, upper and lower jaws
Fibrous dysplasia of the skull bones is often monoosal and localized in the occipital and frontal parts. It does not cause any special problems for the patient and is diagnosed randomly on X-rays.
The expressed fibrous dysplasia of the skull in the tumor-like form can be seen with the naked eye. Such cases are typical for elderly patients. These external centers of deformation of bone trabeculae can be surgically removed.
Relapses occur rarely.
Fibrous dysplasia of the bones of the jaws
Simple radiographic images are used for diagnosis.
In the defeat of tubular bones, it is important to fix the condition of the metaphysis and diaphysis.
On X-ray, fibrous bone dysplasia looks like a clouded glass with clear boundaries of the focus. Densitometry is important for differential diagnosis.
Treatment of fibrous bone dysplasia
Treatment of fibrous dysplasia can only be performed surgically. To date, there are no effective pharmacological agents that could stop or reverse the process of replacing bone trabeculae with fibrous fibers.
In the surgical treatment of fibrous bone dysplasia, a partial resection (removal) of the damaged areas is carried out. Then the cavity is filled with a special compound, which after hardening replaces the bone.
A source: http://santebone.ru/?p=3316