Varieties of fibrous dysplasia and methods of treatment. Fibrous dysplasia of the tibia Fibrous dysplasia of one bone is it treated

​(Visited 876 times, 1 visits today)​ Rapid increase in the pathological focus

The greater trochanter is located above the usual level, approaching the iliac wing

Change in the shape of the bones

Classification

​Recurrence rate of fibrous dysplasia, even after curettage and bone grafting, is high. However, most solitary lesions stabilize with skeletal maturity. As a rule, the single form does not transform into the plural.​

This abnormal protein stimulates G1 cyclic adenosine monophosphate (AMP) and osteoblasts (cells) to a higher rate of DNA synthesis than in normal cells. This leads to the formation of a fibrous disorganized bone matrix with the formation of a primitive bone tissue, which is not able to mature into lamellar bones. The mineralization process itself is also abnormal.

• ​It should be borne in mind that fibrous dysplasia, especially monoosseous dysplasia, can present significant diagnostic difficulties. With mild clinical symptoms, long-term follow-up is often required. To exclude other diseases and assess the state of various organs and systems, consultations with a phthisiatrician, oncologist, therapist, cardiologist, endocrinologist and other specialists may be required. The disease was first described in the first half of the 20th century. In 1927, the Russian surgeon Braytsov made a report on the clinical, microscopic and radiological signs of focal fibrous bone degeneration. In 1937, Albright described multifocal fibrous dysplasia associated with endocrine disorders and characteristic skin changes. In the same year, Albrecht described multifocal dysplasia in combination with precocious puberty and indistinct skin pigmentation. And a little later, Jaffe and Lichtenstein investigated single-focal lesions and published conclusions about the causes of their occurrence. In the literature, fibrous dysplasia may be referred to as Liechtenstein's disease, Liechtenstein-Jaffe disease, or Liechtenstein-Braitsov disease.​
• Treatment of pathological bone formation consists in the use of standard orthopedic measures: strengthening the muscular system, preventing fractures, strengthening the skeleton, etc.
• With the formation of a focus in the fibula, there is no deformity of the limb, with damage tibia there may be a saber curvature of the lower leg or a slowdown in bone growth in length. Shortening is usually less pronounced than with a focus in the femur. Fibrous dysplasia of the ilium and ischium causes deformity of the pelvic ring. This, in turn, negatively affects the spine, causing posture disorders, scoliosis or kyphosis. The situation is aggravated if the process simultaneously affects the hip and pelvic bones, since in such cases the axis of the body is even more disturbed, and the load on the spine increases.
• ​Resistant pain syndrome associated with fibrous osteodysplasia An increase in bone tubercles in the skull - frontal, occipital and parietal
• Lameness (if the process affects the bones of the lower extremities, which leads to their shortening or lengthening)​Multiple forms can be very severe (in their presentation), but they also (most often) stabilize at puberty. However, existing deformities may progress.​
• Clinical photograph of distinctive pigmentation in patients with endocrine disorders associated with fibrous dysplasia. Treatment is predominantly surgical - complete resection of the affected bone within healthy tissues and replacement of the defect with a bone graft. With a pathological fracture, the Ilizarov apparatus is applied. 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 polyostotic form, severe crippling deformities may occur. Sometimes dysplasia foci degenerate into benign tumors (giant cell tumor or non-ossifying fibroma). In adults, several cases of malignant transformation into osteogenic sarcoma have been described.

Symptoms of fibrous dysplasia

There are two main forms of fibrous dysplasia: monoosseous (affecting one bone) and polyosseous (affecting several bones, usually located on one side of the body). The polyossal form develops in childhood and can be combined with endocrine disorders and skin melanosis (Albright's syndrome). The monoosseous form can manifest at any age, endocrinopathies and skin pigmentation are not observed in patients.

- one bone is affected. It occurs in people of all ages. The elderly are especially susceptible to pathology, more often observed in men than women.

The presence of a fracture of a certain localization, in which there is a huge threat to the life of the patient

Increasing the thickness of the temporal arch

Pain at the site of injury

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Fibrous bone dysplasia: modern treatment of severe pathology

For the first time, fibrous dysplasia of the bone was reported in 1927 at the 19th Congress of Surgeons of Russia. that report belonged to V.R. Braitsev, whose name this disease is now called. But it was not always so. Although Braitsev was the first to describe fibrous bone dysplasia, for some time it became known as Liechtenstein-Jaffe disease. These two scientists only supplemented the description given by Braitsev. Subsequently, on the initiative of T.P. Vinogradova in 1973, this pathology was renamed Braitsev-Lichtenstein disease in the entire world literature.

Lytic lesions of the femoral neck with surrounding foci of sclerosis

What is fibrous osteodysplasia?

Fibrous dysplasia is a disease that is characterized by the replacement of bone tissue with fibrous tissue, which causes deformation of the bones.

Surgical intervention should be resorted to only with extensive deformities, when drug treatment not effective.​

Oligoossal form

Causes of the disease

The presence of focal cysts.

Flattening of the bridge of the nose

Pathological fractures.

According to the discoverer, the cause of the development of osteofibrous dysplasia was considered to be a disrupted work of the mesenchyme, which reproduces bone tissue of the fibrous type. As a result, the latter has a completed and incomplete structure. Therefore, fibrosis of the bone marrow occurs with a tendency to its growth and the formation of a typical osteoid. Such disorders develop even in the embryonic period under the influence of uncertain factors. They can spread either to a section of the bone, or to the entire bone, or to involve several bones in a row.

Three-dimensional (3D) reconstruction of a patient's CT scan. Note the expansion of the left maxilla with displacement of the teeth, alveolar process, and superior orbit. The infraorbital rim is also shifted forward.

The reasons for the development of this type of dysplasia have not been fully studied and are completely clear. Today it is generally accepted that this disease there are tumor-like processes associated with impaired development of the osteogenic mesenchyme. Fibrous dysplasia very often begins to develop in childhood, but its occurrence in adulthood and old age is not excluded. Danan pathology is more common in females.

Disease classification

Intraosseous form

Prosthetics are prescribed for pronounced pathology.

1. - damage to two or three bones. As a rule, it affects certain adjacent areas of the skeleton (for example, adjacent ribs or bones of the skull). It should be borne in mind that fibrous dysplasia, especially monoosseous, can present significant diagnostic difficulties. With mild clinical symptoms, long-term follow-up is often required. To exclude other diseases and assess the state of various organs and systems, consultations of a phthisiatrician, oncologist, therapist, cardiologist, endocrinologist and other specialists may be required.​
2. The disease was first described in the first half of the 20th century. In 1927, the Russian surgeon Braytsov made a report on the clinical, microscopic and radiological signs of focal fibrous bone degeneration. In 1937, Albright described multifocal fibrous dysplasia associated with endocrine disorders and characteristic skin changes. In the same year, Albrecht described multifocal dysplasia in combination with precocious puberty and indistinct skin pigmentation. And a little later, Jaffe and Lichtenstein investigated single-focal lesions and published conclusions about the causes of their occurrence. In the literature, fibrous dysplasia can be found under the names of Liechtenstein's disease, Liechtenstein-Jaffe's disease or Liechtenstein-Braitsov's disease. The main stages of surgical treatment are as follows:
3. Shortening of the bones of the upper and / or lower limbs Pain syndrome in fibrous bone dysplasia is characterized by certain signs. These are the following:
4. To date, there is no single classification of fibrous bone dysplasia that would satisfy all the requirements of clinicians. Various options for the division of this pathology are proposed. The most common are the following.​

Depending on the existing spread of the pathological process, it is customary to distinguish two main forms of fibrous dysplasia:

Main symptoms

. May be monoossial or polyossal. Single or multiple foci are formed in the bone fibrous tissue, in some cases, bone degeneration is observed throughout, however, the structure of the cortical layer is preserved, so there are no deformations.

Untimely access to a doctor provokes the following complications:

Polyosseous shape Treatment is predominantly surgical - complete resection of the affected area of ​​the bone within healthy tissues and replacement of the defect with a bone graft. With a pathological fracture, the Ilizarov apparatus is applied. 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 polyostotic form, severe crippling deformities may occur. Sometimes dysplasia foci degenerate into benign tumors (giant cell tumor or non-ossifying fibroma). In adults, several cases of malignant transformation into osteogenic sarcoma have been described.

1. There are two main forms of fibrous dysplasia: monoosseous (affecting one bone) and polyosseous (affecting several bones, usually located on one side of the body). The polyostotic form develops in childhood and can be combined with endocrine disorders and skin melanosis (Albright's syndrome). The monoosseous form can manifest at any age, endocrinopathies and skin pigmentation are not observed in patients. Removal of the bone area affected by the pathological process
2. Mottled inclusions on the general background of the bone Most often characteristic of damage to the bones of the lower extremities and skull, practically absent if the upper limbs or trunk bones are involved in the pathological process
3. Depending on the number of bones involved, the disease is divided into the following types: Patients with small solitary lesions may not feel any symptoms at all and show no signs, and their bone pathologies may be incidentally detected during x-ray examinations for completely different reasons. However, bone pain, swelling and tenderness are the most common symptoms and manifestations in symptomatic patients.​

Mono-ossal form. Only one bone is affected. This form of fibrous dysplasia can occur at any age.

Total bone loss

Therapy options

More than three bones affected. In this case, most often, large extraosseous changes are noticed. The absence of extraskeletal lesions is also observed.

A person can walk, write and control his body at will, thanks to the musculoskeletal system. The skeleton plays an important role in making the human body strong and reliable.

Russian specialists use the clinical classification of Zatsepin, which includes the following forms of the disease:

The consequences of advanced pathology

​Deleting content​

1. A characteristic feature is "frosted glass", which is determined in the picture of the affected bone. Pain has a different duration and, accordingly, the degree of limitation of the patient also varies significantly
2. Monoostic - the process affects only one bone Endocrine abnormalities may be the initial manifestations in some patients. Polyosseous shape. With this form, several bones on one side of the body are simultaneously affected at once. This form of dysplasia can be combined with melanosis. skin and various endocrine pathologies. The polyostotic form occurs in childhood, therefore, patients have a pronounced diffuse deformation of the bones of the skeleton and a predisposition to fractures.
3. . All elements suffer, including the cortical layer and the area of ​​the medullary canal. Due to the total lesion, deformations gradually form, and fatigue fractures often occur. Usually there is a polyosseous lesion of long tubular bones - tumors, chondrosarcomas, osteosarcomas, fibrosarcomas.

Albright Syndrome

Therefore, it is so important to know about diseases associated with the skeletal system.

Intraosseous form

Filling the resulting cavity with a homograft

​Practically complete absence bone marrow canal, which is associated with pathological proliferation of imperfect bone

In the absence of physical activity and during rest, pain practically does not bother a person

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Axial - the long bones of the tubular structure of one of the limbs are affected, for example, the arms or legs The most common skeletal sites at which solitary fibrous dysplasia develops include: ribs, proximal femur, craniofacial bones, and usually the posterior maxilla. These solitary lesions usually cover only a small segment of the bone, but in some cases, this fibrous tissue can take up the entire length of the bone.​

With monoosseous fibrous dysplasia, the ribs are predominantly affected, as well as the shoulder blades, long tubular bones and bones of the skull. In the polyostotic form of dysplasia, more than half of the bones of the skeleton are affected, mainly on one side. Lesions may cover small areas or large parts of the bone. In tubular bones, they are usually localized in the diaphysis, including also the metaphysis. Affected bones at the beginning of the development of the disease retain their shape and size. Then there are foci of "bloating", deformation of the bone tissue, its lengthening or shortening. On the cuts of the bones, strictly limited foci of white color, which have reddish blotches, are determined. These foci are usually round or elongated, sometimes merging with each other. In this case, the medullary canal of the bones is expanded or filled with newly formed tissues.

tumor form

Classification

Against the background of the disease develops

- bone damage is accompanied by skin pigmentation, endocrine disorders. In some cases, girls experience early puberty.

• Even though it's enough rare disease, it has been studied by scientists from all over the world. Having heard from one doctor the diagnosis - fibrous osteodysplasia, from another - Braitsev-Lichtenstein disease, from the third - Jeffie-Lichtenstein pathology, you know, they are talking about the same disease. May be monoossial or polyossal. Single or multiple foci of fibrous tissue are formed in the bone, in some cases bone degeneration is observed throughout, however, the structure of the cortical layer is preserved, so there are no deformations.
• A long period of immobilization of the limb for the formation of a strong bone callus. Alternation of foci of enlightenment with foci of compaction.
• An increase in pain is observed if a person makes any movements or lifts weights Unilateral - bones on one side are affected, for example, the bones of the right upper and lower limbs
• In multiple fibrous dysplasia, the disease can develop from at least two bones to more than 75% of the bones of the skeleton. This form of fibrous dysplasia is most commonly found in the hip, lower leg, pelvis, and leg bones. Other places where this disease also develops, but less frequently, include: the ribs, the skull, and the bones of the upper extremities. And even more rarely (and even unusually), this form of the disease can develop in the lumbar, cervical spine and collarbones. Fibrous dysplasia is currently not treatable. However, it is possible to reduce it clinical symptoms through various orthopedic procedures, such as osteotomy, curettage and bone grafting. Indications for these interventions are progressive deformities, multiple, long-term or nonunion fractures, and pain syndrome, which is not relieved by the use of drugs. With a common form of the disease, accompanied by an increased content of alkaline phosphatases, the use of calcitonin may be effective.
• . Accompanied by the growth of foci of fibrous tissue, which sometimes reach a significant size. Rarely detected. Concomitant pathology
• Often, patients are unaware of the presence of pathologies in them. V initial stage the disease develops without subjective symptoms or clinical objective signs. The ability to convert osteoblastic mesenchyme into bone tissue is lost.

Symptoms of fibrous dysplasia

Total bone loss

In childhood, radical surgery is most often used. It aims to completely limit the spread of this disease. Further bone growth is provided by the stimulation of osteoblasts. If false joints are formed, regardless of the age of the patient, then the Iliazarov apparatus is used. They help prevent limb shortening.

The presence of certain radiological signs of the disease depends on the duration of the disease and the number of bones involved in the pathological process. It is not at all necessary to have a combination of all the above symptoms in one patient.

The presence of lameness and deformity contributes to increased pain.

Bilateral - the bones of the right and left sides are affected.

Diagnosis and treatment of fibrous dysplasia

The most common physical deformities of this disease are leg length discrepancies, facial asymmetries, and rib deformities. Fracture is the most common complication of all two types of fibrous dysplasia. Bone fractures are recorded in more than half of patients. Many individuals also develop deformities of the weight-bearing bones. Almost 75% of patients with fibrous dysplasia suffer from pain, bone deformities, or pathological fractures.​

The most common complication is pathological bone fractures. Especially often there is a fracture of the femur. At the same time, fractures of the bones of the upper extremities are rare. Usually, such fractures heal well, but the deformation of the bones increases.

Albright Syndrome

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Fibrous dysplasia - treatment, diagnosis, symptoms, causes

- impaired hearing, vision, migraines appear, rickets develop, heart rhythm is disturbed.

Etiology and pathogenesis of fibrous dysplasia

Signs of pathology include:

Classification of fibrous dysplasia

In patients, bone tissue is converted into a kind of fibrous substance. The simplest bone structures change dramatically, they contain cartilage inclusions or osteoid substances. An unusual bone is formed with fibrous marrow.

• . All elements suffer, including the cortical layer and the area of ​​the medullary canal. Due to the total lesion, deformations gradually form, and fatigue fractures often occur. Usually there is a polyosseous lesion of long tubular bones.
• Fibrous bone dysplasia is treated by a surgeon or a traumatologist, depending on the level of specialization of the medical institution.

Pathological picture in fibrous dysplasia

​Diagnosis of fibrous osteodysplasia should be timely, as well as subsequent treatment, since this disease belongs to precancerous processes. The following signs bring it together with oncological conditions:

Methods of treatment of fibrous dysplasia

The lesion of the tibia, which is a favorite localization of fibrous osteodysplasia, leads to its curvature anteriorly, as well as to the side. At the same time, lateral flattening of the bone and areas of uneven expansion are observed. Pain can occur either simultaneously with the onset of the deformity, or some time before or after its onset.

Complications of fibrous dysplasia

He does not distinguish between the following forms:

malignant transformation

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​The information provided is not a recommendation for the treatment of fibrous dysplasia, but is a brief description of the problem for the purpose of familiarization. Do not forget that self-medication can harm your health. If signs of illness appear or are suspected, you should immediately consult a doctor. Be healthy.​

Gigantism and acromegaly

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In the photo, fibrous dysplasia of the bones of the skull In the affected areas of the skeleton, instead of bone tissue, a dense, elastic pulpy formation appears. A loose mass containing spindle-shaped cells instead of a lamellar structure provokes the presence of peculiar islands of cartilage tissue.

tumor form

fibrous dysplasia. Epidemiology

Fibrous bone dysplasia is a systemic disease of the skeleton, a violation of the development of bone tissue, although it is not a true tumor, it is close to neoplasms. Among the various neoplasms of the skeleton, it occupies 10% in children. It was first described by Braitsev and Lichtenstein. In areas at the stage of incomplete calcification, there are accumulations of osteogenic fibrous tissue. Being a malformation of the bone, like cartilage dysplasia, fibrous bone dysplasia is observed more often in children and adolescents.

Possibility of transformation of fibrous osteodysplasia into a true tumor, especially in childhood

A change in the shape of the femur also leads to a characteristic deformity. It consists in a pronounced curvature of the thigh to the outside. In this case, the greater trochanter can reach almost the iliac wing. This leads to a significant change in gait. Such a deformity has various names in the medical literature, for example, the shape of a hockey stick, the handle of a jug, etc.

fibrous dysplasia. Causes

Albright syndrome - characteristic bone lesions in which there is increased skin pigmentation and puberty in girls, which begins earlier than the age norm

​Malignant transformation of fibrous dysplasia is very rare, with a reported incidence of approximately 0.4-4%. More than half of patients with malignant transformation underwent radiation therapy.​

fibrous dysplasia. Photo

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Fibrocartilaginous dysplasia

- increased activity of growth hormone.

Bone deformity

fibrous dysplasia. Symptoms and manifestations

In some cases, the presence of individual huge cells such as osteoclasts. Fibrous tissue can migrate from the epiphyseal plate, spread and cause osteoid tissue, forming bone with an incomplete type of bone formation.

. Accompanied by the growth of foci of fibrous tissue, which sometimes reach a significant size. Rarely seen.

When large areas of the bone are affected, the first symptom of the disease may be fractures (in 30% of cases), which subsequently recur. Sometimes the disease attracts attention due to the resulting deformities, the appearance of swelling of the bone, curvature of the axis of the limb, slowing down the growth of the bone in length.

Relapse of the pathological process after surgical removal the focus of the lesion

A pathological process that affects the humerus causes it to expand like a club. At the same time, the structure of the hand is also disturbed, the appearance of which resembles chopped off fingers. This happens due to the shortening and thickening of the finger phalanges.

Polyosseous - only bones are involved in the process, with 2 or more

The most common malignant tumors are osteosarcomas, fibrosarcomas, and chondrosarcomas. Most patients are > 30 years of age. Most of these cancerous tumors develop in the bones of the skull, followed by those of the hips, lower legs, and pelvis. The rate of malignant transformation is higher in single lesions than in multiple ones.​

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fibrous dysplasia. Diagnostics

. It appears as a predominant degeneration of cartilage, degeneration into chondrosarcoma is often observed.

Doctors' prognosis is often disappointing. Although, in most cases, this is not a fatal disease, it often leads to disability.

. The curvature can be barely noticeable or significant (up to a change in the entire segment of the limb). Thickening or damage to the bones depends on how long the disease lasts and its localization. In some cases, this symptom does not accompany pain. In others, it appears with him, or much later.

​The question of the origin of fibrous bone dysplasia is still a matter of debate in the medical scientific community.​

Albright Syndrome

fibrous dysplasia. Treatment

Localization of fibrous dysplasia of bones is diverse. In practice, the lesion can be in any bone. Both diffuse and focal lesions are located in the metaphyses and diaphyses, more often in the long tubular bones of the extremities. With diffuse forms, the lesion of the subtrochanteric region of the thigh predominates, with multifocal monoosseous - the diaphysis of the tibia. The possible symmetry of the lesion and the predominant unilateral lesion of all segments of the limb speak in favor of the congenital nature of the disease.

Some features of morphology

Bone curvature causes loose joints. This creates conditions for the development of a degenerative-dystrophic process in them (deforming osteoarthritis), which further disrupts the human condition. The situation can be aggravated with incorrectly fused fractures, in which the bone is finally bent and shortened.

fibrous dysplasia. Forecast

Monoosseous - only one of any bones is affected.

Plain X-ray

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Calcifying fibroma

Classification

Fibrous dysplasia of the tibia

pathological fractures

• Despite the variety of interpretations, most scientists stick around towards the theory of the pathogenesis of the disease. Considering it as a congenital malformation of bone tissue.
• . It manifests itself as a polyosseous or almost generalized bone lesion in combination with endocrine disorders, premature puberty in girls, a violation of body proportions, focal skin pigmentation, severe deformities of the bones of the trunk and limbs. It is accompanied by progressive disorders from various organs and systems.
• Fibrous bone dysplasia is sometimes combined with other diseases. Jaffe lists among them the combinations he observed with hyperthyroidism, a rudimentary kidney, congenital atrophy of the optic nerve, and also with multiple fibromyxoid tissue tumors. All these examples speak of a dysontogenetic basis with impaired mesenchymal development. The so-called Albright syndrome is also a confirmation of this thought.​
• Malignancy, that is, malignancy of the process.

With fibrous bone dysplasia, rarer forms of the disease can also occur, in which the following anatomical structures are affected:

• ​In modern medicine The most rational is considered to be a classification based on clinical and radiological data. According to her, the following forms are distinguished:
• ​When X-rays are taken, machine operators and other competent specialists may detect fibrous dysplasia as an intramedullary and well-defined focus in the diaphysis or metaphysis of the bone. Lesions can range from fully translucent to fully sclerotic. However, most lesions have a characteristic hazy or ground glass appearance. The degree of opacification on the x-ray correlates directly with its underlying histopathology. More radiolucent lesions are composed predominantly of fibrous elements, while more radiopaque lesions contain a greater proportion of bone tissue. In addition, the lesions may be surrounded by a thick layer of sclerotic reactive tissue, which is called the "peel."

• Against the background of frequent fractures, the natural fixation of the skeleton is disrupted, the overall proportion of the body structure is disrupted.
• . When the bones grow together, degenerative-dystrophic zones of restructuring are formed. Frequent pain. There may be a fracture of the bone, with intraosseous hemorrhage, occurring even in the absence of overload.
• I consider the doctrine of the dysplastic nature of the development of fibrous osteodysplasia to be the most modern and widespread, considering the likelihood of transforming underdeveloped bone tissue into a tumor.
• Fibrocartilaginous dysplasia
• The reasons for the combination of symptoms remain unclear, but they speak in favor of a dysplastic nature. The fact that gonadotropic hormone enters the blood suggests that the endocrine system is involved secondarily: apparently, bone changes of a dysplastic nature also affect the bones of the base of the skull.

Symptoms

Thus, Braitsev-Liechtenstein disease is a dysplastic process in which there is a real possibility of fully undeveloped bone tissue into a tumor tissue with pathologically rapid growth and division.

• Calls
• Monoosseous - the disease involves only one bone
• ​Scintigraphy, CT and MRI​
• is a non-inherited bone developmental anomaly in which normal bone tissue is replaced by fibrous bone tissue. This condition was first described in 1942 by Lichtenstein and Jaffe. Hence, fibrous dysplasia is sometimes referred to as Liechtenstein-Jaffe disease. The disease process can be localized in one or more bones.

• At the same time, with single or small foci of fibrous bone dysplasia, the prognosis is positive. It is not dangerous and does not pose a threat.​
• Lameness
• Violation of the functioning of the endocrine, central nervous system, or system: pituitary ¬¬- adrenal cortex, as clinical observations have shown, are the main cause of the disease.
• . It appears as a predominant degeneration of cartilage, degeneration into chondrosarcoma is often observed.
• Microscopically, the foci of the disease are poorly differentiated osteogenic tissue. Osteoblasts, or rather fibroblasts, cellular fibrous tissue are involved in the formation of fibers and bone beams, but their structure is extremely primitive and they do not allow the formation of solid bone substance.

The treatment of fibrous dysplasia of the bones is associated with various difficulties. The biggest of them is that there are no means of pathogenetic therapy, since the disease is associated with a violation of embryonic development.

Ribs

Oligoossal - 2 or 3 bones

​Scintigraphy can be used to determine the extent of the disease. Active fibrous lesions, especially in young patients, significantly increase isotope uptake, this uptake will become less intense as the lesion matures.​

​Localized fibrous dysplasia can occur as part of McCune-Albright or Mazabraud syndrome. Fibrous dysplasia can also develop in association with other endocrine dysfunctions, such as hyperthyroidism, hyperparathyroidism, acromegaly, diabetes mellitus, Cushing's syndrome.​

• ​Constituting approximately 5.5% of all bone diseases and 9.5% of benign bone tumors (according to data provided by M.V. Volkov), the disease nevertheless deserves closer attention. Every year more and more children and the elderly get sick of her.
• . It is caused by a change in the length of the limbs (lengthening or shortening). There are pains in the affected area.

​Disturbances result from the natural, but distorted and extremely slow, pathological development of the embryonic osteoblastic mesenchyme.​

Diagnostics

Calcifying fibroma

• Great difficulties for the differential diagnosis of fibrous dysplasia of the bone are regional focal forms of education. At the marginal location, dysplasia may resemble giant cell tumors, foci of eosinophilic granuloma in the stage of repair, and bone abscesses.
• In addition, the causative factors of this pathology are not completely known. Thus, Schlumberger believed that fibrous osteodiplasia was based on perverse reactions of bone tissue to traumatic injury. Not all patients have osteoblasts that function normally to fully compensate for the resulting defect in the bone. Someone has the formation of immature tissue, which is associated with the development of the disease. Some authors have tried to explain what is happening pathological changes as a result of malfunctioning of the endocrine system. In confirmation, they cited Albright's syndrome. However, endocrine disorders were found not in all patients. Recklinghausen considered this pathology as a local manifestation of neurofibromatosis.
• Pelvic bones.
• Polyosseous, within which forms are distinguished without extraosseous manifestations and forms with such, for example, an incomplete version of Albright's syndrome
• But best of all, the extent of the lesion is demonstrated on computed tomography. On CT, in most patients fibrous dysplasia can be easily distinguished from other lesions in the differential diagnosis.​
• Fibrous dysplasia accounts for about 5% of all benign bone lesions. However, the true incidence is unknown, as many patients are asymptomatic. Localized fibrous dysplasia accounts for 75-80% of cases.​
• The bones of the lower extremities are bent under the weight of the body, characteristic deformations occur. The femur is especially sharply deformed, in half of the cases its shortening is detected. Due to the progressive curvature of the proximal parts, the bone takes the form of a boomerang (shepherd's crook, hockey stick), the greater trochanter "shifts" upward, sometimes reaching the level of the pelvic bones. The femoral neck is deformed, lameness occurs. Shortening of the hip can be from 1 to 10 cm.​
• Improperly formed bone organs reduce the supporting function of the skeleton. Such an anomaly is accompanied not only by bone deformity, but also by frequent fractures. With multiple localization, it can lead to disability.
• Hormonal disorders
• Cells lose their ability to transform into normal osteoblasts.

. A special form of fibrous dysplasia, very rare, usually affecting the tibia.

The prognosis for life in children is favorable. At the same time, it is possible to transfer the focus to benign tumor Non-ossifying fibroma or giant cell tumor of the bone. In addition, cases of malignant transformation of the focus into osteogenic sarcoma in adults are described. That is why children should be under the direct supervision of a doctor. As for the functional prognosis, large bone lesions - diffuse and multifocal forms in one bone, in the absence of orthopedic prophylaxis and treatment can lead to significant mutilation: bone curvature, protruding deformities, multiple pathological fractures, and in the diffuse form - to their long-term fusion, up to the formation of false joints. Small solitary foci of fibrous dysplasia are not dangerous. In the absence of concerns, they should not be removed based on self-ossification.​

• Currently, some conditional concept has been adopted regarding the causes and mechanisms of the development of this disease. It is believed that this is an embryonic disorder that occurs after birth due to the presence of a congenital neurological disorder. This is confirmed by the presence of central disturbances in the system of the pituitary gland and the adrenal cortex. This circumstance may open up new possibilities for treatment (the use of certain pharmacological drugs with endocrine activity).​
• One feature of fibrous osteodysplasia is important to note. the disease always begins in childhood, then its progression is observed (slow or rapid, depending on the action of causative factors). At the onset of puberty, stabilization of the pathological process is observed. Therefore, treatment of this disease should begin as early as possible in order to keep as many bones as normal as possible.
• Albright Syndrome
• Asymptomatic, radiologically characteristic fibrous lesions require only clinical observation. X-rays should be taken every 6 months. If new lesions are identified in the patient, doctors will need to exclude the diagnosis of the multiple form of this disease. Otherwise, the patient should be referred to an endocrinologist for early detection of possible systemic disorders.​

Fibrous dysplasia is a slow growing lesion that usually appears during periods of bone growth and thus can often be seen in individuals in early adolescence and late adulthood. adolescence. Fibrous dysplasia, which develops in a few bones, accounts for 20-25% of all cases, and patients with this form tend to show symptoms at a slightly earlier age (mean age 8 years).​

Treatment

When a focus is formed in the fibula, there is no deformity of the limb; if the tibia is affected, a saber-shaped curvature of the lower leg or a slowdown in bone growth in length can be observed. Shortening is usually less pronounced than with a focus in the femur. Fibrous dysplasia of the ilium and ischium causes deformity of the pelvic ring. This, in turn, negatively affects the spine, causing posture disorders, scoliosis or kyphosis. The situation is aggravated if the process simultaneously affects the hip and pelvic bones, since in such cases the axis of the body is even more disturbed, and the load on the spine increases.

. Landcard-shaped or freckled brown spots on the skin of the buttocks, neck of the neck, etc. Adenoma is often observed thyroid gland, hyperthyroidism. For girls - early development sexual characteristics, premature menstruation. In boys - excessively rapid growth, early coarsening of the voice, etc.

The process of skeletal ossification that occurs at the time of embryonic formation is extremely anomalous. The child begins to get sick in the womb, during the primary formation of bone organs, although symptoms may not appear immediately after birth.

Severe congenital deformities are usually absent. In the polyosseous form, the first symptoms appear in childhood. Bone damage is accompanied by endocrinopathies, skin pigmentation and disruption of the cardiovascular system. The 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 when a pathological fracture occurs.

• Treatment is surgical only. Shown resection of the lesion within healthy tissues, no matter how large it may be, with the replacement of the defect with homografts.
• The main task in the treatment of such patients is to reduce the severity of the manifestations of this pathology and improve the possibility of self-service and performance professional activity. To accomplish this task, orthopedic treatment is used in most cases. Measures to prevent pathologically occurring fractures are also very important. In this case, tactics will be determined by bone mineral density, calcium and phosphorus levels in the blood.
• Diagnosis of fibrous bone dysplasia is largely based on the results of x-ray examination. Its implementation in combination with an assessment of clinical manifestations dramatically reduces the percentage of diagnostic errors. The main distinguishing features of this disease, which can be identified on the radiograph, are the following:
• A mixed form, in which not only bone tissue is involved in the pathological process, but also cartilaginous (bone-cartilaginous dysplasia).

Bisphosphonates, and primarily pamidronate, may be used to reduce bone pain in symptomatic patients with solitary fibrous dysplasia. An open biopsy may be indicated for those patients who will show uncharacteristic signs. Most patients will require surgery to correct deformities, prevent pathologic fractures, or remove symptomatic lesions.​

• Pregnancy can cause increased growth of the lesion. Males and females develop the disorder equally, although the multiple variant that is associated with McCune-Albright syndrome is most common in women.​
• The monoosseous form proceeds more favorably, there are no extraosseous pathological manifestations. The severity and nature of the deformities vary greatly depending on the location, size of the focus and the characteristics of the lesion (total or intraosseous). There may be pain, lameness and fatigue after loading the affected segment. As with the polyostotic form, pathological fractures are possible.
• - a bone lesion in which a section of normal bone tissue is replaced by connective tissue with the inclusion of bone trabeculae. Fibrous dysplasia belongs to the category of tumor-like diseases, it can be local or widespread, affecting one or more bones. The reasons for the development are not clear, a genetic predisposition is not ruled out. Manifested by pain, deformity, shortening or lengthening of the segment and pathological fractures. The diagnosis is made on the basis of radiography, MRI, CT and other studies. Treatment is usually surgical.
• The paucity of clinical symptoms complicates the process of diagnosing the disease on early stages development. In this case, laboratory tests will not show deviations, since metabolic processes are not violated.​

​The prognosis and treatments depend largely on the number of bones affected.​

Which doctor treats

Usually, in the polyostotic form, there is a lesion of the tubular bones: tibia, femur, fibula, humerus, radius and ulna. Of the flat bones, the bones of the pelvis, the bones of the skull, the spine, the ribs and the scapula are most often affected. Often, damage to the bones of the hands and feet is detected, while the bones of the wrist remain intact. The degree of deformation depends on the localization of foci of fibrous dysplasia. When a process occurs in the tubular bones of the upper extremities, only their club-shaped expansion is usually observed. When the phalanges are affected, the fingers shorten, look "chopped off."

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Fibrous bone dysplasia - Oncology

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Symptoms of fibrous dysplasia of the bone

Surgical treatments for fibrous skeletal dysplasia are not routine. For their use, there are certain indications when the likelihood of obtaining the most favorable result is high. The main indications are:

Varus deviation of the thigh, that is, more towards the outer surface

The course of the disease in different cases may be different. For unknown reasons, some patients have a slow course, while others have a rapid progression of the pathological process. Such rapid growth is combined with pronounced cellular polymorphism, which brings it closer to the oncological process (with bone sarcoma). Most often, the tibia is involved in the pathological process, which leads to a characteristic appearance patient. However, the character clinical picture depends on the location of the lesion. Common clinical symptoms are as follows:

​Treatment for malignant transformation should be based on the subtype of sarcoma, but the prognosis is generally poor.​

Fibrous dysplasia is caused by a somatic mutation in the GNAS1 gene located on chromosome 20q13.2-13.3. This gene encodes the alpha subunit of the G stimulatory protein, Gsα. Due to this mutation, the amino acid arginine (in the protein) at position 201 (R201) is replaced by the amino acid cysteine ​​(R201C) or histidine (R201H).​

The diagnosis is made on the basis of the clinical picture and X-ray data. At the initial stage, X-rays in the area of ​​the diaphysis or metaphysis of the affected bone reveal areas that look like frosted glass. Then the affected area acquires a characteristic speckled appearance: foci of compaction alternate with areas of enlightenment. The deformation is clearly visible. If a single focus is detected, it is necessary to exclude multiple bone lesions, which in the initial stages may be asymptomatic, so patients are referred for densitometry. If there are suspicious areas, x-rays are performed, if necessary, bone CT is used.

Prognosis for fibrous dysplasia of the bone

Fibrous dysplasia is a systemic skeletal lesion that belongs to the category of tumor-like diseases, but is not a true bone tumor. Occurs as a result of improper development of osteogenic mesenchyme (tissue from which bone is subsequently formed). Symptoms are usually detected in childhood, but late onset is also possible. The literature describes cases when monoosseous fibrous dysplasia was first diagnosed in people of retirement age. Women get sick more often than men. Perhaps degeneration into a benign tumor; malignancy is rare.

Treatment of fibrous dysplasia of the bone

Fibrous dysplasia of the bones is detected by the results of an X-ray examination. In the picture, doctors can notice a zone of diffuse enlightenment of the bone tissue. Diagnosis must be timely, as well as treatment, since this disease is classified as a precancerous process.

There are four forms of the disease according to the number of bones affected by the disease: The bones of the lower extremities are bent under the weight of the body, characteristic deformations occur. The femur is especially sharply deformed, in half of the cases its shortening is detected. Due to the progressive curvature of the proximal parts, the bone takes the form of a boomerang (shepherd's crook, hockey stick), the greater trochanter "shifts" upward, sometimes reaching the level of the pelvic bones. The femoral neck is deformed, lameness occurs. Shortening of the hip can be from 1 to 10 cm.​

The word dysplasia means abnormal development. From the Greek dys - disturbance, disorder, plasseo - I form, sculpt, build.

What developmental disorder? Any tissue: cartilage, bone, connective, muscle. Any organ.

In relation to the joints, it means the incorrect location of the bones in the joint, the violation of the comparison of the articular surfaces (congruence), the irregular shape of the bones that form the joint. The irregular shape of the bones of the joint also changes the external shape of the joint.

Behind the diagnosis lies a violation in the development of cartilaginous tissues, it mainly develops during the intrauterine formation of the fetus. Another name for dysplasia is congenital dislocation of the hip. In fact, this is a change in the normal joint, which involves the deformation of the femoral head along with the articular surface of the pelvic bone - the acetabulum.

This disease is most often detected at birth or during infancy, however, it happens that dysplasia is also detected in adulthood. In women, the violation is detected 2 times more often than in men. It also increases the likelihood that a mother suffering from this disease will have a child with a similar pathology.

Dysplasia hip joint(DTS) is a developmental pathology of the joint that can cause hip dislocation. It is expressed in the wrong proportion of the size of the femoral head and the acetabulum (the recess where it is located).

The synonymous name that was used earlier to describe this congenital problem has the same name - congenital hip dislocation. Now it is customary to use a different name, since the root cause of the disease is dysplasia, that is, the entire process of development of the constituent parts of the joint is disrupted during the period of human physical development.

The presence of the term "dysplasia" in the name of the disease indicates that this problem appeared even before the birth of a person.

Statistics show that the disease occurs in many countries. The disease is associated with environmental factors, some traditions of swaddling, and even with racial and ethnic characteristics of the body.

Since in the last decades of the twentieth century the classification of the disease has been changed and its diagnosis has been refined, the statistics have risen significantly. This is due to the fact that doctors began to attribute such pathologies as preluxation and subluxation to hip dysplasia.

By international classification diseases of the tenth revision, hip dysplasia has the code Q65 (Congenital deformities of the hips).

CAUSES

Internal causes:

• family predisposition to dysplasia
• hereditary changes

factors affecting a woman's body during pregnancy

• physical and chemical agents
• some medicines(a group of tetracycline antibiotics)
• harmful factors in production
• bad ecology
• bad habits
• exposure to poisons, toxins
• viral diseases during pregnancy
• toxicosis of pregnancy
• malnutrition, with a lack of minerals, vitamins.

As a result of exposure to harmful factors during pregnancy, there is a violation of the development, formation of bones, ligaments and muscles of the knee joint.

The exact cause of the congenital anomaly has not been identified. It is associated with the development of joints at 5-6 weeks of intrauterine development of the child, and is often detected when the child begins to walk. As for adults, it is rare, but it happens: joint dysplasia can be formed due to injury.

The greatest possibility of developing negative variants is with the following problems:

1. Underdeveloped tissues of muscles, cartilage and ligaments.
2. Injury to the hip joint during childbirth.
3. Breech, breech presentation.
4. An increased content of the hormone progesterone and relaxin in the mother during pregnancy, as a result - a weakening muscle tissue and sacro-femoral joints.
5. Incorrectly or not fully developed acetabulum.
6. Large fetus at birth.
7. Underdeveloped motor function of the child.

The main cause of the disease in adults is incorrect or insufficient treatment in childhood. It is possible that in infancy the pathology was not detected at all, then the treatment becomes more complicated.

The hip joint consists of the acetabulum and the femoral head, which are lined with cartilage. The acetabulum, femoral head, ligaments and cartilage form the articular bag, or capsule, which is filled with joint fluid. In newborns, this joint differs in shape from the adult joint in that the cavity is less deep, the ligaments are more elastic.

Normally, the head should exactly fit the cavity and not pop out of it. With pathological development, the cavity becomes flat and decreases in size, the femoral neck shortens, so the head pops out and makes it difficult to move the joint or, conversely, makes it too mobile.

The angle of the femur, which has a head at the base and ends with the condyles of the femur, may be disturbed.

One of the reasons for the development of dysplasia in adulthood is tight swaddling in early childhood, as a result of which the femoral axis is displaced and the head of the bone is outside the acetabulum.

Joint dysplasia in an adult occurs, as a rule, with a special anatomical structure mobile connections. Often there is a congenital dislocation of the hip joint, which later leads to pathology. The following causes influence the development of residual joint dysplasia in women and men:

• Genetic failures, due to which bones, movable joints and ligaments are not properly formed.
• Trauma in which the hip joint is damaged. Often, pathology is noted during pregnancy and in the process of labor.
• External negative factors, such as constant stress, the influence of toxins, infectious lesions.
• Excessive physical activity, in which pressure on the pelvic region increases.
• Hormonal disorder in the body. A common cause of the violation is pregnancy, as well as the period of menopause and menopause.
• Abuse of alcohol, tobacco products.

Reliable reasons for the development of knee dysplasia are still unclear. A huge role is played by burdened heredity and the influence of damaging factors in the prenatal period.

The true causes of hip dysplasia are still unknown. The predisposing factors are as follows:

• underdevelopment of the acetabulum;
• the inclusion of new mechanisms in the movement of the joint during the beginning of walking.

Approximately in 2-3% of cases, dislocation is formed already in utero.

As mentioned above, hip dysplasia is detected already in early childhood during routine visits to an orthopedic doctor. Suspicion can cause the following symptoms:

• excessive rotation of the hip;
• shortening of the lower limbs, one or two at once;
• when moving in the area of ​​the hip joint, it is noted as if slipping or clicking;
• if you bend your legs at an angle of 90º in the hip and knee joints and try to spread them, incomplete abduction of the limbs is noted;
• asymmetrical arrangement of skin folds.

Clinical picture

Symptoms of fibrous dysplasia may vary depending on the location of the lesion. In some patients, the pathology develops slowly, in others - quickly. The reasons for the discrepancy are still not clear to doctors.

With the rapid growth of pathological tissue, strong cell polymorphism occurs. This feature of dysplasia is similar to the development of a malignant tumor.

The main types of fibrous dysplasia are monoosseous and polyosseous. The first form indicates that only one bone is affected, and the second - several bone tissues nearby. The polyostotic type is diagnosed more often in children. Often accompanied by a malfunction of the endocrine organs, melanosis of the skin.

A monoosseous type of pathology is found at any age without any additional disorders.

Doctors use the following classification fibrotic diseases bones:

1. Intraosseous view. It affects both one and several bones, inside which foci of replacement by connective tissue are formed. Sometimes the transformation of the entire bone with the exception of the cortical layer is noticeable.
2. Total view. It affects all components of the tissue, causing the development of bone deformity, which leads to fractures. More often than not, multiple lesions are diagnosed.
3. tumor type. It is combined with the growth of fibrous tissue. Sometimes neoplasms become quite large. This disease is extremely rare.
4. Albright Syndrome. With this type of pathology, several bones are affected. In humans, endocrine system disorders, premature puberty in girls, changes in the size of body parts, age spots on the skin, and severe deformation of bone tissue are also detected. The patient may be disturbed by various diseases internal organs.
5. Fibrocartilaginous appearance. With such a violation, cartilage tissue is replaced, which often leads to degeneration into chondrosarcoma.
6. An evocative look. Rarely diagnosed. Affects the tibia of the lower limb of a person.

Disease of the femur

The femur is the largest among the elements of the skeleton, as a result of which a significant load is placed on it. Because of this, with the development of fibrous dysplasia, the bone is deformed, and it often shortens.

The hip bone during the pathological process is deformed so much that it looks like a hockey stick. The proximal part of the tissue is subjected to curvature so significantly that the greater trochanter is located at the level of the pelvis.

As a result of such processes, the femoral neck is deformed, patients begin to limp, which creates a large load on the skeleton. With fibrous lesions, shortening can be both small (1 cm) and pronounced (over 10 cm).

• Acetabular. The cavity of the mobile joint becomes flat, and the limbus of the vertebra becomes thinner and shifts.
• Changes in the hip head. When deviated, the overgrown bone tissue forms outgrowths (osteophytes).
• Rotary. This type of dysplasia is characterized by simultaneous damage to the hip and knee joints, due to which lower limb unfolds to the inside.

Varieties and severity

After the patient voices complaints and confirms the symptoms, an X-ray examination is prescribed. The pictures clearly show violations, and it is possible to establish not only the presence of the disease, but also the stage of development.

There are three stages of the disease:

1. Predislocation - when the hip joint is underdeveloped, while there is no displacement of the head of the bone from the acetabulum.
2. Subluxation - in addition to deformity of the joint, the head of the femur is displaced a small distance from the cavity.
3. Dislocation is the last stage in the development of the disorder. The head is strongly displaced in relation to the acetabulum.

The possibility of negative consequences and the speed of recovery depend on the neglect of the disease. In adult patients, the course of treatment is difficult.

Orthopedists distinguish three degrees of development of DTS, which are characterized by various forms of the disease:

• First degree dysplasia in the form of preluxation

At the first degree, the first symptomatology of the problem is the impossibility of breeding the child's legs, bent at right angles at the knees and hips. This happens due to the fact that the child has increased muscle tone in the areas around the joints, so complete dilution is impossible.

It can be expressed to varying degrees due to the localization of the center of the femoral head. Other signs that you should pay attention to are the asymmetry of the skin folds on the buttocks and thighs.

• Second degree dysplasia in the form of subluxation

The second degree is clearly defined by the symptom of slippage, when the head cannot be held in the acetabulum. When entering and leaving it, a characteristic push and a small sound are felt, indicating that the head goes over the edge of the bed.

This is due to the fact that its back part is not completely developed and formed. The leg can be located in the outward position, and also be slightly shorter when flexing for the second.

At the same time, changes in the pattern of skin folds are present.

• Third degree dysplasia in the form of dislocation

Coxarthrosis is a degenerative-dystrophic pathology that affects the hip joint. Most often it is detected in patients older than 50 years, but very young people also suffer from it.

Coxarthrosis is characterized by gradual development over several years. The main causes of joint destruction are injuries, pathologies of an inflammatory or degenerative nature: arthritis, osteoarthritis, and various infections.

There is no 4th degree of coxarthrosis in the medical classification. Grade 3 is considered the most severe, accompanied by irreversible damage to the hip joint. 4 x-ray stage of the disease - complete or partial fusion of the joint space. In this state of articulation, the lower body of the patient loses mobility.

Conservative treatment is aimed only at eliminating the symptoms. The main method of therapy is endoprosthetics.

The mechanism of development of pathology and the main causes

In orthopedic and traumatological practice, grade 4 coxarthrosis of the hip joint is the most common disease among all diagnosed arthrosis. This is due to the increased load on the joint.

The defeat of this joint (congenital dysplasia) is most often found in newborns. Representatives of both sexes suffer from coxarthrosis, but women are more susceptible to the destruction of the joint of a woman.

In a normal state, the surface of the iliac and femoral bones is smooth and even. When moving, the structural elements of the joint are displaced relative to each other.

Smooth sliding is provided by a thick fluid, a small amount of which is produced in the synovial bag. In a healthy person, hyaline cartilage is smooth, elastic, strong, and correctly distributes loads during movement.

This well-coordinated process is disrupted under the influence of external and internal negative factors:

• causes of secondary coxarthrosis are dysplasia, congenital dislocations, Perthes disease, aseptic necrosis of the femoral head. Pathology is provoked by infectious lesions and inflammatory processes, traumatic dislocations, fractures;
• The causes of primary coxarthrosis have not yet been established. Scientists suggest that circulatory disorders or increased stress can lead to its development. For example, premature destruction of the joint contributes to lifting weights, including during sports.

Coxarthrosis of the 4th degree usually develops in the absence of medical intervention at the initial stage. One-sided pathology is more often diagnosed, but it can also be bilateral, the most severe.

The disease is not considered hereditary, but certain features can be passed on to the child from the parents. This is a predisposition to metabolic disorders, a certain structure of cartilaginous tissues or the structure of the spinal column.

For coxarthrosis of the 4th degree, excessive viscosity of the synovial fluid, which loses its depreciation properties, is characteristic. Hyaline cartilage becomes dry, rough, cracks appear on its surface.

The bones are deformed due to improper distribution of loads and grow together. Movement in the hip joint in this state is impossible.

Muscles strongly atrophy, stretch, lose elasticity.

Factors provoking primary coxarthrosis	The main causes of secondary coxarthrosis
Increased physical activity - strength sports, lifting and moving weights, overweight	Injury to the joint - fractures, dislocations, subluxations, ruptures of ligaments and tendons or their complete separation from the base of the bone, strong blows, prolonged pressure
Circulatory disorders, insufficient intake of nutrients and biologically active substances into the hip joint	Chronic articular or bone pathologies - osteoporosis, osteoarthritis, rheumatoid, juvenile, psoriatic, gouty arthritis, ankylosing spondylitis
Elderly or old age, when collagen production decreases and ligaments and tendons weaken	Infection of the articular cavity with staphylococci, streptococci, Mycobacterium tuberculosis, pathogens of syphilis, gonorrhea, brucellosis

DTS in children

Children with a developmental pathology such as hip dysplasia need immediate treatment, since in only 3% of cases there is an inability to cure this problem. But even these 3% are subject to certain adjustments, because of which many serious consequences can be avoided.

If the treatment of a child with dysplasia is delayed until the second half of the year, then the cure rate becomes only 30%. Therefore, it is very important to identify the disease before 6 months. This can be done, both by the parents of the child, and should be done by pediatricians examining him.

The sooner a problem is detected, the shorter the treatment time will be. It is important to note that in cases where it is detected before three months, it will take only two more to solve it. In the event that parents notice dysplasia after the first year until the body is fully formed, which occurs at about 20 years old, certain measures and methods of treatment will have to be resorted to.

Interestingly, experts note a direct relationship between the methods of swaddling a child and the possibility of the origin of congenital hip dislocation. The tighter this is done, aligning the child's legs, the more likely it is to happen.

Statistics confirm this, including the policy adopted in Japan to abolish the tradition of tight swaddling, which has significantly reduced the incidence of this problem.

Rehabilitation after hip dysplasia in adults

To reduce the risk of complications, it is required to comply with all medical prescriptions during rehabilitation. If an adult treats dysplasia operational way, then after the manipulation for some time it is worth refraining from physical exertion.

Only with the permission of the doctor do a little gymnastics at a moderate pace. Physiotherapeutic procedures are also carried out to improve blood flow and normalize the metabolic process.

Often, rehabilitation includes taking painkillers and anti-inflammatory drugs. Helps to recover faster swimming and massage.

Main symptoms

The rate of treatment of the disease largely depends on how quickly it is detected. An early diagnosis can be made based on the symptoms of the disease. According to statistics, in most cases, hip dysplasia affects left leg.

The main symptomatology, which speaks of a possible hip dysplasia, manifests itself in infancy:

• acute pain even at rest;
• when turning the leg, a characteristic click or crunch is heard;
• shortening of the diseased limb;
• instability in a static position;
• lameness.

When these symptoms appear, diagnostics are carried out in order to accurately establish the diagnosis.

In order to diagnose congenital hip dysplasia, which has not yet led to hip dislocation, it is necessary to examine a child of childhood in a state of relaxation. Therefore, this is often done in a calm and quiet environment during the feeding period.

Some clinical tests may indicate an orthopedic problem, among which experts distinguish:

• Asymmetric skin folds.
• Shortening of one of the hips.
• Marx-Ortolani slip symptom.
• Impossibility or limited possible retraction of the hip to the side.

The indicators of all tests are taken into account, since if there is a problem, for example, on two legs, asymmetry may not be manifested. This also applies to the age of the child, since at the age of two or three months this symptom may be more pronounced than at birth.

The difference in the folds of the skin is at what levels they are, what is the difference in depth between them, as well as the shape. Pediatricians during the examination pay attention to the gluteal, popliteal and inguinal fold features of the skin.

In case the child has a problem, they are deeper and there are more of them to a large extent. But because this symptom is not common in half of patients, it is not a clear indication that a problem exists.

One of the most reliable tests that indicates the most severe form of the disease is hip shortness. In the prone position with the same bending of both knees, the bottom of them is below the second. This is due to the fact that the femoral head is displaced from behind, depending on the acetabulum.

The slipping symptom, which was independently described by two scientists in the 1930s, is a characteristic indicator of the presence of hip dysplasia. To do this, you need to take the child's hips to the sides, and then carefully monitor how they will behave.

In this case, it turns out that the femoral head enters the acetabulum, where it behaves more tightly and slowly, while there is a quick push as it exits it. In the first weeks of life, positive test does not always indicate the presence of the disease.

There are some indirect symptoms that may not confirm the disease, but make a certain alertness to the examination. Among them are:

1. Softness of the bones of the skull.
2. Krivoshei.
3. Polydactyly.
4. Flat heel valgus or valgus foot placement.
5. Violation of some reflexes (search, sucking and neck tonic).

To identify mild degrees of development of the disease, it is possible to carry out radiographic diagnosis with its subsequent description by an experienced specialist.

• impaired gait, limping when walking;
• different leg lengths;
• clicks or crunching when moving;
• pain during the load on the movable joint;
• dystrophic changes in cartilaginous tissue.

Often, with dysplasia, a dislocation of the hip joint occurs in adults, which is associated with a stretched capsule and a violation in the ligamentous apparatus.

The diagnosis is established by a specialist on the basis of a clinical examination, X-ray and MRI of the knee.

In early childhood, dysplasia is often asymptomatic.

With the onset of walking in children, the manifestations of knee dysplasia become quite pronounced. Lameness, instability of body position with frequent falls, uncertainty of walking are noted. There is a loss of balance when squatting. When the knee moves, characteristic "clicks" are heard.

Flexion contracture of the knee joint develops with limited stride length, outward rotation of the lower leg, valgus deformity of the knee and hindfoot.

Diagnosis and symptoms

In case of pathology, they turn to an orthopedist or rheumatologist. The doctor will collect a complete medical history and prescribe additional diagnostic manipulations, on the basis of which an accurate diagnosis is established. Mostly dysplasia is detected through instrumental methods, but sometimes laboratory tests are required. Necessary procedures in case of rejection:

• Ultrasound of the hip area;
• x-ray, which reveals the underdevelopment of the acetabulum;
• CT and MRI as additional manipulations.

TREATMENT

Basically, therapy is carried out in early childhood, but the treatment of pathology is also possible at an older age, although it is much more difficult to achieve complete recovery of older patients.

Methods of treatment are divided into surgical and conservative. Conservative treatment is possible only if the disease has not passed to the very last stage, and the joint deformity does not have an extremely neglected appearance.

Non-surgical treatments include:

1. Relieve pain with medications. Oral medications can also relieve inflammation.
2. Intravenous injections to improve blood circulation in the joint are used if the vessels are damaged, as well as to increase tissue nutrition.
3. Physiotherapy for warming up and removing swelling in the focus of the disease.
4. Therapeutic massage and physical education.
5. Wearing orthopedic appliances.
6. Closed reposition.

A complex of gymnastic exercises for dysplasia can greatly improve the patient's condition. It is necessary to select gymnastics that helps to treat the disease with an experienced specialist, based on individual indicators.

Basically, the complex includes such exercises:

• flexion and extension of both legs alternately with the same load;
• correcting the position of the foot while walking;
• massage.

All exercises for dysplasia must be done, carefully listening to your own feelings, and at the slightest pain, pause or simplify and reduce the load. The regularity of the sessions is very important.

If such methods of treatment do not show effective results for a long time, an operation is prescribed.

Surgery in the treatment of dysplasia

There are several types of operations to treat the disease. There is a certain way for every situation. Therefore, the doctor, before prescribing treatment, must make sure that more gentle methods do not help, it is necessary to accurately determine the stage of the disease and give specific recommendations. Types of surgery:

1. The most used method is considered to be open reduction of the dislocation. The doctor makes his way to the hip joint of the patient under anesthesia, adjusts the head so that it takes the correct position. Sometimes this requires deepening of the acetabulum. After such an operation, a plaster bandage is applied to the patient.
2. An osteotomy is a type of surgery to change the shape of the bones. Surgical intervention is used as an opportunity to correct the patient's femoral head or acetabulum.
3. Another method of surgical intervention is the creation of a kind of “roof” on the pelvic bone, which will not allow the head to move even more.

If other methods are not possible, a partial or complete replacement of the joint with an artificial prosthesis is used. Such a decision is made when the disease has serious complications, the restoration of the joint is impossible.

Methods and methods of treating different types of diseases are slightly different from each other. It is necessary to make a decision based on clear clinical manifestations of dysplasia, as well as a comprehensive diagnosis of the disease. Treatment can be conservative or radical surgery. The second method is used only in the most severe cases.

In conservative treatment, various technologies and methods for eliminating the problem are used, including:

1. Conducting a wide swaddling, which allows you to maintain the activity of the joints and not restrict the movement of the limbs.
2. Establishment of special spacers that allow you to remove the legs of the child.
3. Impact by applying special plaster bandages that fix the legs in certain poses.
4. Physiotherapeutic methods. Physiotherapy for hip dysplasia can include many techniques aimed at reducing the activity of inflammation, improving trophic processes in the muscles, preventing the formation of contractures and reducing pain. In this case, electrophoresis, amplipulse therapy, ultrasound, mud treatment, magnetic laser therapy, hyperbaric oxygenation, therapeutic massages and acupuncture are used.

It is important to monitor the health of your child from an early age. You can find out how hip dysplasia is treated in children under one year old, what are the symptoms of this dangerous disease.

In this video, you can learn how to massage yourself for both a child and an adult.

In the case of indications for surgery, one of two solutions can be taken, among which are open surgery on the joint in order to reduce it, or endoscopic treatment, which does not leave significant marks on the body and is much less radical.

To access the joint during endoscopy, only a few small incisions are made, through which they pass to the area that needs to be corrected.

After the treatment, it is mandatory to undergo rehabilitation, selected in accordance with the age of the child and the degree of development of the disease that has been eliminated.

For any problems with the joints, especially in a child, it is necessary to contact specialists, who may be surgeons or orthopedists. Self-treatment may not lead to the desired result, delay the further treatment process, or even aggravate the situation.

If the treatment and detection of the disease is timely, the situation can be corrected completely or, in some cases, partially. This will significantly improve the lifestyle of a person and increase his opportunities.

With a mild course, it is possible to cure dysplasia in adults using conservative methods. During therapy, osteopathy is used, which allows to normalize the functions of the musculoskeletal system and improve the condition.

An osteopath with hip dysplasia performs a special massage that improves blood flow. It is impossible to perform such procedures on your own, since it is possible to harm and aggravate dysplasia.

But if the pathology has become more complicated, and coxarthrosis has developed, then one massage is not enough, additional therapeutic measures are required:

• Reception of chondroprotectors and non-steroidal anti-inflammatory drugs that are administered intravenously or taken orally.
• The use of muscle relaxants that relieve spasms.
• The use of drugs to improve blood flow.

Physiotherapeutic treatment of dysplasia in adults is absolutely necessary, in which healing baths and salt heating pads are prescribed. Applications with paraffin are performed, which act specifically on the damaged area. Complex therapy also includes vitamins and minerals, thanks to which a person will recover faster, strengthen the joints and the body as a whole.

Comprehensive treatment of hip dysplasia must be started as early as possible. short time both in children and adults. Massage is the first treatment.

It is carried out only by a trained person, especially when treating children. The minimum massage course is 10 days, but the duration depends on the severity of the pathological process.

An effective method of conservative treatment is the wearing of special underwear, bandages or orthopedic corsets.

An orthopedic surgeon treats hip dysplasia.

With the development of complications of hip dysplasia in the form of coxarthrosis, medications:

1. To relieve swelling and inflammation of the cartilage and soft tissues, non-steroidal anti-inflammatory drugs are prescribed in the form of injections and in the form of tablets or rectal suppositories.
2. Relieve spasm skeletal muscles, reduce pain syndrome allow muscle relaxants.
3. In order to improve blood circulation, eliminate stagnation and edema, vascular preparations are prescribed.
4. With a pronounced inflammatory process, especially of an aseptic or autoimmune nature, steroid hormones with a pronounced anti-inflammatory effect are prescribed.
5. To restore the structure of cartilage tissue, long-term courses of chondroprotectors are prescribed.
6. Local anti-inflammatory drugs.

Drug treatment does not belong to the main group, only eliminating symptoms in case of complications.

The main treatment includes physiotherapy and exercise therapy. Physiotherapy treatment involves the appointment of therapeutic baths, using heat to improve blood circulation in the joint. Paraffin applications have a good therapeutic effect. They act purposefully on diseased areas, are effective in children and adult patients.

Therapeutic massage improves the trophism of cartilage and ligamentous tissues, tone and blood circulation in the muscles. Conservative treatment is provided long-term, regular and targeted. During treatment in children, the functions of the hip joint are restored quickly, without consequences.

Conservative and surgical methods are used to treat knee dysplasia.

Treatment of hip dysplasia must be carried out from the moment the disease is discovered. Massage therapy is usually used as a therapy.

Massage is carried out only by a specialist, in order to avoid injury to the child. The course of treatment depends on the degree of dysplasia, in some cases ten days is enough.

In other situations, in addition to massage, you will need to wear a special corset until complete recovery.

But there are cases when the pathology is missed, not detected for one reason or another in childhood. Then serious consequences await already an adult.

After all, hip dysplasia in adults in such cases is the result of an untreated or not completely cured disease in childhood. Therefore, it is necessary to start treatment on time, to work only with qualified specialists in order to avoid serious consequences.

The consequences of childhood dysplasia can be expressed in the development of diseases such as neoarthrosis or coxarthrosis in adults.

Treatment of knee dysplasia should be carried out with the help of wide swaddling of a newborn child. To do this, a triple folded diaper is placed in the crotch area, which is fixed on top with a tight swaddling.

Thus, the child's legs are constantly in a fixed position, which makes it possible to restore the cartilaginous joints in the area of ​​the patella. Pronounced dysplastic changes are eliminated by using a plaster cast, which fixes the limbs in an inflexible position.

For children older than two years who are diagnosed with dysplastic changes in the knee joint area, surgical intervention is indicated. After the operation, it is necessary to carry out strengthening procedures in the form of massage for some time, which stimulate the muscles and restore the joint bag.

COMPLICATIONS AND PROGNOSIS

Even with proper treatment and noticeable improvements in the functioning of the patient's musculoskeletal organs, complete recovery after such an injury is impossible. But, in addition to direct negative consequences, complications can also develop, especially with hip dysplasia in adults, since the disease was discovered rather late.

The most common are the following consequences of pathology:

• scoliosis;
• lordosis;
• osteochondrosis;
• flat feet;
• kyphosis;
• decrease in resistance to long-term loads of the physical plane.

With hip dysplasia, the development of quite serious illnesses requiring treatment.

One of the consequences of the disease can be neoarthrosis. The disease consists in the fact that a new joint grows in the region of the acetabulum or on the head of the femur, which is not able to perform all the functions of a real joint. Useless and interfering education is removed surgically.

If blood vessels are damaged, this process contributes to the manifestation of necrosis of the femoral head due to a violation of the supply of nutrients, blood, and with it oxygen. The head is destroyed and cannot be naturally restored. This can be corrected only with the help of prosthetics.

Coxarthrosis develops for a long time, it is a more frequent complication of hip dysplasia, manifested in adults. In fact, coxarthrosis is arthrosis, in which cartilage and articular bones are destroyed. If coxarthrosis is started, complete destruction will occur without the possibility of recovery.

Hip dysplasia in adults is a serious disease that is difficult to treat. Even after effective treatment and return to normal life, the patient must undergo an annual preventive examination by specialists.

Of course, it is best to identify this pathology in childhood, for this it is necessary to carefully conduct examinations in infants, and parents to notice all possible violations in order to determine congenital hip dislocation in time.

With timely detection of the disease and treatment, the patient leads a normal life, the disease does not return.

If people in adulthood are not diagnosed in a timely manner with dysplasia of the left hip joint or damage to the right movable joint, then there is a high probability of developing serious complications that affect health. Without treatment, the following consequences threaten:

• flat feet;
• disorders in the spinal column that provoke scoliosis and osteochondrosis;
• dysplastic coxarthrosis;
• aseptic necrosis of the head of the movable hip joint.

Disease prevention

Due to the fact that the exact causes of the development of fibrous bone dysplasia are unknown, doctors cannot develop special preventive measures.

Every expectant mother should know about preventive measures to prevent the acquisition of such a diagnosis as knee dysplasia.

After all, the health of a newborn child depends on her lifestyle and nutrition.

From the first months, the child needs to have massage sessions, and also make sure that his position in the hands of adults is correct. Be sure to hold the back of the baby, and avoid sitting astride the hip of an adult.

Knee dysplasia makes it impossible to walk beautifully, developing in the patient not only complexes and a decrease in self-esteem, but also dangerous consequences for other organs.

Therefore, only early diagnosis of the disease and its treatment can help correct the situation, avoid complex surgical procedures and long recovery.

• Preventive examination by a neurologist and orthopedist;
• At the slightest suspicion of dysplasia, it is necessary to conduct an ultrasound of the joints;
• Developing physical education and preventive massage;
• Proper holding of the child in his arms - back support is required, it is not recommended to seat the baby "on top" on the side of an adult.

megan92 2 weeks ago

Tell me, who is struggling with pain in the joints? My knees hurt terribly ((I drink painkillers, but I understand that I am struggling with the consequence, and not with the cause ... Nifiga does not help!

Daria 2 weeks ago

I struggled with my sore joints for several years until I read this article by some Chinese doctor. And for a long time I forgot about the "incurable" joints. Such are the things

megan92 13 days ago

Daria 12 days ago

megan92, so I wrote in my first comment) Well, I'll duplicate it, it's not difficult for me, catch - link to professor's article.

Sonya 10 days ago

Isn't this a divorce? Why the Internet sell ah?

Yulek26 10 days ago

Sonya, what country do you live in? .. They sell on the Internet, because shops and pharmacies set their margins brutal. In addition, payment is only after receipt, that is, they first looked, checked and only then paid. Yes, and now everything is sold on the Internet - from clothes to TVs, furniture and cars.

Editorial response 10 days ago

Sonya, hello. This drug for the treatment of joints is really not sold through the pharmacy network in order to avoid inflated prices. Currently, you can only order Official website. Be healthy!

Sonya 10 days ago

Sorry, I didn't notice at first the information about the cash on delivery. Then, it's OK! Everything is in order - exactly, if payment upon receipt. Thanks a lot!!))

Margo 8 days ago

Has anyone tried folk methods joint treatment? Grandmother does not trust pills, the poor woman has been suffering from pain for many years ...

Andrew a week ago

What kind of folk remedies I have not tried, nothing helped, it only got worse ...

39819 0

As you know, science and history can not only forget, but also remember.

In 1927 V.R. Braitsev at the 19th Congress of Russian Surgeons was the first to give a detailed description of the clinical, radiological, microscopic picture of altered bones, and reported on the microscopic structure of the focus of fibrous dysplasia. He believed that the basis of the disease is "deviation of the functions of the osteoblastic mesenchyme ... Osteoblastic mesenchyme creates a bone of an incomplete structure." Therefore, one must agree with the opinion of T.P. Vinogradova (1973), which is much more reason to assign the name of V.R. to fibrous bone dysplasia. Braitsev than to call it Liechtenstein's disease or Liechtenstein-Yaffe's disease, which only clarified and further developed the provisions of V.R. Braitsev.

V.R. Braitsev also described fibrous dysplasia in the journals New Surgery (1928) and Archive klinische Chirurgi (1928), i.e. 10 years earlier than J. Lichtenstein, who in 1938 reported this disease, and in 1942 described 15 of his own observations.

It is necessary to correct this historical injustice: the merit of V.R. Braitseva in the discovery of a new nosological unit - fibrous bone dysplasia - is obvious.

V.R. Braitsev in 1927 at the XIX Congress of Russian Surgeons also made a report on local osteodystrophy - osteodystrophia fibrosa localisata (cystica). He said: “Surgeons are especially interested in the practical side of the matter, but the expediency of surgical measures for local osteodystrophy can only be based on complete clarity of the nature of the disease.” Knowing well the world literature on this issue, he, on the basis of three of his own observations, puts forward a new original theory of the origin, essence of the disease, singles it out as an independent nosological unit, substantiates the causes of the development of fibrous tissue of cysts and recommends methods of treatment. The description of the morphological picture of fibrous dysplasia is unusually accurate. In conclusion, he draws the following conclusions.

1. The essence of fibrous osteodystrophy is a functional deviation of the osteoblastic mesenchyme during bone development in the embryonic period, as a result of which a kind of bone with fibrous bone marrow is created from the very beginning, capable of growing and giving "osteoid tissue and bone of an incomplete type."

2. Such a deviation in the function of the osteoblastic mesenchyme can occur in isolated areas of a single bone, can spread to the whole bone and even to many bones of the skeleton.

3. The growth of fibrous tissue is active, but the energy of growth in different cases is different. In some cases, the process proceeds quietly, slowly, in others - rapidly, accompanied by a large polymorphism of cells, which morphologically brings it closer to sarcomatous.

4. Solitary bone cysts, according to data obtained by many authors, develop on the basis of osteodystrophia fibrosa due to edema and liquefaction of the central fibrous growths, and also, possibly, on the basis of hemorrhages in the fibrous tissue.

V. R. Braytsev recommended performing subperiosteal resection throughout with replacement of the defect with autografts, because “pathological fibrous tissue, as can be seen from the histological examination, penetrates the bone membrane up to the periosteum.”

In the debate on his report, such prominent surgeons as I.I. Grekov, S.P. Fedorov, N.N. Petrov, but it is clear from their speeches that they underestimated the unique data obtained by V.R. Braitsev, - the discovery of a new nosological unit by him. All these surgeons, like N.N. Terebinsky and T.N. Krasnobaev, spoke only about bone cysts, which they encountered frequently and for which they sometimes performed operations.

Under our supervision in the hospital there were 245 patients with fibrous dysplasia; the number of patients with polyostotic lesions was significantly greater than the number of patients with monoosseous process requiring surgical treatment.

According to the literature, monoosseous and polyosseous forms of fibrous dysplasia are observed almost equally often, however, according to M.K. Klimova (1970), nevertheless, the polyosseous form is somewhat more common, and M.V. Volkov (1968, 1985).

Clinic. With sharp deformations of the skeleton, patients are rarely born. Symptoms of fibrous dysplasia usually appear in childhood and are characterized by diversity: it is either minor pain sensations more often in the hips, or the appearance of deformity and its increase, or a pathological fracture due to severe and inadequate trauma, while the correct diagnosis is not always made.

In the polyostotic form of fibrous dysplasia, the tibia, femur, fibula, humerus, radius, and ulna are most often affected. The frequency of lesions (in decreasing order) of flat bones: pelvic bones, skull bones, vertebrae, ribs, scapula. The bones of the foot and hand (but not the bones of the wrist) are relatively often affected.

Children with Albright syndrome are sometimes born with severe deformities and, of course, with a typical age spot. After the first manifestation of the disease, both clinical and radiological signs can progress and the intraosseous form of the disease can then go into a form with damage to the entire cortical layer or in the area of ​​one of the foci, most often in the upper end of the femur or throughout the entire diaphysis, which indicates different activity of the dysplastic process. The epiphyses of the bones, as a rule, are not affected. The progression of the process in children and young people is often accompanied by fractures. According to A.I. Snetkov (1984), in a patient operated on at the age of 4 (marginal resection, removal of fibrous tissue, bone alloplasty), after 7 years, a restructuring of allografts with zones of their lysis caused by the growth of new pathological foci was observed. Thus, there is a programming in the development of dysplasia: dysplastic fibrous tissue develops in some patients in areas of the bone that previously seemed normal radiographically.

L.N. Furtseva et al. (1991) revealed significant disorders of the glucocorticoid function of the adrenal cortex in patients with fibrous dysplasia: “The level of calcium in all types of the disease is increased, but not in proportion to the extent of bone tissue damage; at the same time calcium excretion with urine is reduced in comparison with the norm. The decrease is more pronounced in the polyosseous form than in the monoossous form. With limited forms of the disease, phosphaturia is reduced, with extensive lesions of bone tissue, only a downward trend is noted. Total aminazot and total hydroxyproline of urine are increased in extensive processes, and in Albright's syndrome and the polyostotic form with widespread lesions, the excretion of amino acids is significantly higher.

Cardiovascular system: more often in patients with polyosseous form, sinus tachycardia is observed - 96-140 per 1 min, sinus arrhythmia is less often noted on the ECG and in most patients arterial hypotension - 115/60 and even 95/50 mm Hg, with In this case, in some patients, the metabolism in the heart muscle is disturbed. An increase in ESR is noted: in patients with a monoosseous form - up to 15-27 mm/h, with a polyosseous form - up to 22-45 mm/h. When studying the function of the adrenal glands, the content of 11-hydroxycorticosteroids (11-OKS) in plasma was determined; in patients with the polyostotic form, a violation of the functional state of the adrenal cortex was revealed, as evidenced by a less pronounced increase in the level of total and active 11 -OCS in blood plasma and a weakened or paradoxical response to the administration of adrenocorticotropic hormone (ACTH).

One 30-year-old patient with a polyostotic form died during surgery in 1974 from a sudden fall. blood pressure; the second, at the age of 19, died after surgery in 1978 from progressive pulmonary heart failure. In pathoanatomical examination, in addition to typical changes in the bones, similar changes were found: polycystic ovaries, adenomatosis of the cortical layer of the adrenal glands, thyroid gland, anterior pituitary gland and thymus hyperplasia.

A.I. Morozov, V.P. Ivannikov (1972), studying a case of "Albright's disease" in a 16-year-old patient, revealed changes in the deep midline structures of the brain on the electroencephalogram. A.G. Povarinskiy, Z.K. Bystrova, with the help of encephalographic studies conducted in parallel with neurological studies, established deep and characteristic for this disease dysfunction of the brain and instability of cerebral homeostasis as a result of a disorder of regulatory mechanisms located in the area of ​​deep structures. All this made us carefully examine patients with fibrous dysplasia, especially with the polyostotic form, for the last 20 years.

In a number of patients, this dysplasia proceeds hidden. We consulted a professor-surgeon, 62 years old, who for the first time accidentally found fibrous dysplasia of the entire left femur and tibia on radiographs, and did not establish indications for surgical treatment. We do not believe that all patients with fibrous dysplasia should be operated on, especially with the intraosseous form. Indications for surgery are pain caused by progressive deformity, fatigue fractures, the presence of cysts with a sharp thinning of the cortical layer, lameness, shortening of the limb, compression of the spinal cord, etc. In case of a fracture at the site of the cyst, we perform conservative treatment, wait 8 months after fusion, and if the cyst remains the same size or progresses, we operate.

M.V. Volkov (1985) distinguishes between polyosseous, monoosseous and regional forms of fibrous dysplasia, and according to the nature of changes in the bone - focal and diffuse. We offer a clinical classification with more detailed description features of each form, using the concept of "memory of the shape of the bone" proposed by us, however, it should be borne in mind that fibrous dysplasia is a pathological process that has countless variants and transitional forms.

Clinical classification of fibrous bone dysplasia (according to S.T. Zatsepin)

Our proposed classification includes the following forms. I. Intraosseous form of fibrous dysplasia: foci of fibrous tissue can be single, multiple, occupy any part of the bone or bone throughout, however, the cortical layer can be thinned, but retains a normal structure - the shape of the bones remains correct, since there is no memory impairment bone shapes. One bone, bones of various limb segments can be affected, i.e. The process is either monoosseous or polyosseous.

An adequate operation is the complete removal of fibrous tissue by marginal resection of the bone of the required length, removal of fibrous tissue and replacement of the cavity with preserved bone homotransplants. The operation can be considered radical if the walls of the cavity where the fibrous tissue was located are carefully processed with a chisel. With this form, cysts often develop in the center of the fibrous masses; cysts reach the cortical layer and thin it, as a result, pathological fractures often occur (Fig. 15.1). More often they resort to a two-stage tactic: 1) skeletal traction is applied - fragments grow together well, since the cortical layer and periosteum are normal; often cystic cavities disappear, and if not, then - 2) marginal resection, removal of fibrous masses, bone alloplasty, or an operation is performed according to our method (see below).

II. The pathological process captures all elements of the bone: the area of ​​the medullary canal, the cortical layer, spongiosis of the metaphyses, long bones are more often affected throughout, however, the severity of the pathological process varies; it is usually a polyosseous lesion. The defeat of all elements that form the bone (its total defeat), reduces its mechanical strength, leads to gradually advancing deformations, fatigue fractures. With this form of fibrous dysplasia, the SYNDROME OF BONE SHAPE MEMORY DISTURBANCE IS EXPRESSED. There are no adequate radical surgical interventions (except for the removal of the entire affected bone - see section 15.2). Orthopedic corrective osteotomies with alloplasty and metal structures are widely used.

Rice. 15.1. fibrous dysplasia. Intraosseous form. a - part of the focus is occupied by a cyst; b - operation according to S.T. Zatsepin with a repeated fracture: the ends of the fragments are exposed; chisel, spoons, reamers remove fibrous tissue; through the opening of the bone marrow canal at the site of the osteotomy - a fracture; fragments are fixed and the cavity is filled with allogeneic fibula, first introduced proximally, then distally.

Tumor-like growths of foci of fibrous tissue can reach large sizes, which indicates a greater massiveness of the process, but are rarely observed.

III. Tumor forms of fibrous dysplasia.

IV. Albright's syndrome is a special form of dysplasia, when, along with a polyosseous or almost generalized form - total fibrous bone dysplasia - a number of endocrine disorders are observed with early puberty in women, pigmentation fields on the skin, violation of body proportions, often small growth; severe deformities of the bones of the limbs, pelvis, spine, bones of the base of the skull, pronounced changes in the cardiovascular and other body systems. During life, the process progresses, bone deformities gradually increase. The syndrome of impaired memory of the shape of the bone is pronounced.

There are many variants of this form of the disease, not a single patient repeats the other, it is necessarily different in some way. The study of the morphological structure of pathological fibrous and bone tissue in patients with this form of dysplasia showed that various clinical manifestations of this dysplasia correspond to a wide variety of histological patterns, so the task of researchers is to study and clinical and radiological morphological comparison, which will undoubtedly allow to identify subgroups of patients.

V. Fibrocartilaginous bone dysplasia as a special form in our country was identified and described by M.A. Berglezov and N.G. Shulyakovskaya in 1963, who observed a patient with a pronounced clinical and radiological morphological picture. In the described observations, manifestations of cartilaginous dysplasia come to the fore, cases of development of chondrosarcoma are not uncommon.

VI. Calcifying fibroma of long bones refers to a special type of fibrous dysplasia, which was described in 1958 by N.E. Schlitter, R.L. Kempsom (1966), who studied it under an electron microscope.

THEN. Goerghen et al. (1977) presented a description of 2 patients, one of whom relapsed after curettage and resected along with the periosteum for 10 cm, but after 1 year there was a small recurrence at the end of the lower part of the tibia - this was the only observation when the tumor recurred . A total of 8 such tumors were described localized in the tibia, and one tumor in humerus. By histological structure tumors are identical to those observed in the bones of the facial skull and jaws.

Treatment. In patients with fibrous dysplasia, conservative treatment is not used by any of the authors known to us. We were forced to use this treatment in 8 patients with fibrous dysplasia, in which the bones of the limbs, pelvis, spine and most of the ribs were changed. They suffered from severe pain not only when standing upright, but also when breathing. In patients whose 1st, 2nd, 3rd ribs were changed, we could relieve pain by resected the most altered rib or ribs (during surgery, it is important to remember that due to loss of strength, they sink and are located deeper than adjacent normal ribs). When most of the ribs and vertebrae are affected, this cannot be done, and we treat them with repeated courses of calcitonin injections. In all patients, pain decreased, they noted an improvement, but we could not carry out systematic treatment in a large number of patients for a number of years due to the small amount of the drug. Since patients with polyostotic fibrous dysplasia have a variety of changes in the endocrine system, we are confident that it is necessary to develop scientific rationales for conservative treatment. Since in a number of patients with age, some foci, often after a fracture at this level, undergo calcification and ossification, it is necessary to include active metabolites of vitamin D3 and complexones in the complex of conservative treatment.

Operative treatment. V.R. Braytsev (1927) used both marginal resection with defect replacement and subperiosteal resection with replacement of the defect with autografts, since, according to his data, the pathological tissue penetrates the cortical layer to the periosteum. In the following years, after the second "discovery" of this dysplasia, orthopedists began to operate less radically. A typical operation was marginal resection of the cortical layer of the affected bone throughout the entire lesion, i.e. often throughout the entire diaphysis and metaphyses, careful removal of all fibrous masses with a sharp spoon, a semicircular chisel and replacement of the defect with auto-, and for the last 35 years with bone allografts.

Rice. 15.2. Shepherd's stick deformity of the femur.
a — fibrous dysplasia, deformity of the upper half of the femur; b — S.T. Zatsepin’s technique: corrective osteotomy at the apex of the curvature, the cavity is filled with allogeneic fibula and cortical grafts.

In 1978, when correcting a typical varus deformity of the upper part of the femur according to the “shepherd’s stick” type (Fig. 15.2), we became:

1) medially move the upper end of the lower fragment, bringing it under the femoral neck, i.e. shorten the lever to correct the position of the upper fragment, i.e. heads, necks of the greater trochanter;

2) widely dissect the capsule of the hip joint along the upper surface (we have never had to cut off the tendons of the gluteal muscles from the greater trochanter, as A.I. Snetkov (1984) writes about, although we operated on adult patients, and he operated on children and adolescents;

3) subcutaneously cross the tendons of the adductor muscles of the thigh near the pubic bone;

4) remove fibrous masses from the upper and lower fragments;

5) insert the allogeneic fibula into the distal and proximal canal in the form of fragments as an intramedullary fixator and valuable plastic material;

6) additionally fix the fragments with a Trotsenko-Nuzhdin plate.

The following should be specially noted. First, when performing corrective osteotomies in patients with fibrous dysplasia, as a rule, it is better to do a simple transverse osteotomy, rather than a figured one, since the thinned cortical layer breaks when compared and the resulting spikes form an ideal connection. Secondly, on November 13, 1978, in most patients, we refused marginal resections to remove fibrous masses from the bone. After the performed osteotomy to correct the deformity, without marginal resection, we destroy the fibrous tissue inside the bone with reamers to expand the canal with the introduction of intramedullary nails (as is known, their diameter ranges from 6 to 16 mm), and then we remove the fibrous masses with gynecological curettes that have sufficient length. Without violating the walls of the fragments, we have the opportunity to insert the fibula intramedullary sometimes together with a metal nail, obtain reliable fixation and replace the cavity after removal of the fibrous tissue with preserved bone (Fig. 15.3). We believe that if fibrous tissue is completely removed from the bone, then detachment of the periosteum to disrupt the blood supply to the affected bone, recommended by A.P. Berezhny, M.V. Volkov, A.N. Snetkov, is optional.

M.V. Volkov, A.N. Since 1982, Snetkov began to use extra-osseous massive angular metal plates in patients with diffuse lesions of the femur throughout the entire length, including the neck and trochanteric region of the segment, and a massive cortical allograft was placed on the side opposite to the fixator, i.e. they applied the technique and bone fixator (with a slight change) that we proposed and began to successfully apply in 1972 in patients with imperfect osteogenesis. As long-term observations have shown, our method of metal plate fixators and bone grafting, which allowed us to obtain excellent results in osteogenesis imperfecta, in a large number of patients with diffuse forms of fibrous dysplasia gave a temporary effect, and then the femur began to bend, the screws fixing the bone grafting — break or come out of the bone (we had the opportunity to see such patients who were operated on in the children's department, and then came under our supervision in the adult CITO polyclinic).

We see the explanation for this in the fact that with osteogenesis imperfecta, the memory of the shape of the bone is preserved, while with diffuse lesions of the bones with fibrous dysplasia, the bones lose their shape memory and deformities, as a rule, recur to one degree or another.

Therefore, we believe that it is a mistake to call the operations currently being performed radical, as some authors do. It must be acknowledged that currently radical treatment fibrous dysplasia was not found. Sometimes the treatment of such patients presents great difficulties.

Here is an example.
Patient T., 27 years old. Due to sharp deformities of the arms and legs, the parents abandoned their daughter, and her grandmother took her from the maternity hospital. Due to severe changes caused by polyostotic fibrous dysplasia, the patient was bedridden for many years (Fig. 15.4). She was admitted to CITO, where we sequentially performed 5 surgical interventions on both femurs, right tibia, left humerus and both bones of the left forearm. The features that should be emphasized were the use of segmental osteotomies, intramedullary fixation with a CITO nail and plastic with very massive cortical adlografts (2/3 of the femur diameter), which played the role of a biological fixing splint, with which the fragments of the femur were fused along its entire length. This is very important because such massive allografts never completely rebuild and therefore do not curve; bone adhesion over such a large surface is stronger than metal screws.

Rice. 15.3. Sharp deformity of the "shepherd's stick" type


Rice. 15.4. Polyosseous fibrous dysplasia.
a, 6 severe changes and deformities of both femurs and bones of the left tibia and an attempt to correct the deformity (Bogdanov pin, ruler); e - external orthoses; patient 23 years after surgery.

January 18, 1981 — triple corrective osteotomy of the right femur with intramedullary pin fixation, plasty with a massive cortical allograft with a length equal to the diaphysis of the patient's bone. March 28, 1981 — double osteotomy of the left femur, intramedullary pin fixation and similar alloplasty. 06/21/81 - corrective osteotomy of the bones of the left leg in the upper third, removal of fibrous masses, intramedullary fixation, alloplasty. Morphological examination: fibrous dysplasia with a wide layer of osteoid. February 27, 1985 — osteotomy of the left humerus at two levels, removal of pathological tissue, extra- and intramedullary osteosynthesis with allografts. October 31, 1985 — osteotomy of the left ulna, removal of fibrous tissue, autologous alloplasty. Operations were performed by S.T. Zatsepin.

The patient is equipped with orthopedic appliances, works as a correspondent for a local newspaper.

It must be remembered that in some patients with Albright's syndrome, dysplastic processes in the bones progress with age, the deformity of the spine, skull, chest, limb bones increases, and the deformities corrected by operations recur. If during the operation, during the removal of fibrous masses, increased bleeding is observed, this means that such patients have severe bleeding both during and after operations. One patient, operated on by us 4 times, had severe bleeding three times in the postoperative period, for which the surgeon on duty had to operate and stitch the bleeding areas.

We observed malignancy of fibrous dysplasia in 7 patients; we give an example.

Rice. 15.5. Malignancy of the polyostotic form of fibrous dysplasia. The first fracture of the right femur occurred at the age of 6 years. Marginal resection and plasty with cortical allografts were performed twice, followed by femoral lengthening according to Ilizarov. A telangiectatic sarcoma developed. Interiliac-abdominal amputation.

Fedkushov Yu.I., 21 years old. March 6, 1958 — longitudinal partial resection of the upper and middle third of the right thigh with homoplasty. From January 1959 he got the opportunity to walk with a cane. 03/23/60 - longitudinal resection of the cortical layer along the outer surface in the middle and lower third of the right thigh, homoplasty. In 1967 - lengthening of the thigh. Done by G.A. Ilizarov. In 1983, pain appeared, and then the volume increased by 4 cm in the upper and middle third of the right femur. Biopsy: telangiectatic sarcoma (Fig. 15.5). On December 5, 1984, an interiliac-abdominal amputation was performed. Lived for 19 years.

S.T. Zatsepin
Bone pathology in adults

Unfortunately, it has not yet been possible to determine the exact reasons for the changes. Doctors believe that the whole thing is in mutations, but there is no evidence yet.

Doctors additionally identify a number of factors predisposing to fibrosis. Among them:

• various hormonal disorders, especially dangerous in childhood, when bones are just being formed;
• pathologies of the ligamentous-muscular apparatus, due to which the region of the tibia, thigh, jaws often suffers;
• the presence of a genetically fixed predisposition;
• malnutrition of a woman during childbearing (neglect of fruits and vegetables);
• action of factors environment and bad habits
• use of certain medicines during pregnancy;
• severe toxicosis;
• malnutrition syndrome, etc.

However, all these factors only predispose to the development of the disease, but their presence does not lead to it in 100% of cases.

Classification

With the development of fibrous bone dysplasia in children, it is customary to distinguish several forms of the disease. First of all, the division occurs into mono- and polyostotic pathology. In monopathology, only one bone is affected.

With polypathology, several bones are involved in the process, but only on one side of the body. For example, fibrous dysplasia of the tibia, maxilla and skull can be diagnosed, but only on the left or only on the right.

There is another classification in which they distinguish:

• diplasia of the intraosseous type, in which the patient rarely has severe bone deformities, and when examining one or more bones there are foci of fibrosis;
• total type, in which the entire bone as a whole is affected, and the patient has severe deformations of the bone skeleton;
• Albright syndrome - a form of the disease diagnosed in children and characterized by rapid progression;
• fibrocartilaginous form, which is characterized by the ability to often degenerate into a malignant tumor.

Additionally, tumor-like and calcifying forms are distinguished, but they are very rare.

Symptoms

It is important to understand that pronounced congenital deformities are usually absent in this disease. Symptoms of pathology are very diverse, depending on the affected joint. If the hip bones are affected, the patient may complain of mild pain, the deformation will occur gradually. Sometimes the diagnosis is made only after a pathological fracture has been formed.

The polyostotic form entails damage to the tubular bones on one side. Involved more - and fibula, humerus, ulna, radius, femur. Fibrous dysplasia of the bones of the skull and pelvis also occurs in the polyostotic form. Shoulder blade, ribs, spine may be involved. Interestingly, the bones of the wrist are usually not included in the process.

The lower limbs are always bent. This is due to the fact that they are forced to bear the weight of the body. Particular attention is drawn to the shortening of the femur. Depending on the severity of the disease, shortening can reach 10 cm on one side. In this case, the patient begins to limp, complaining of pain in the joint.

Fibrous dysplasia of the knee joint or tibia can lead to the fact that a person's lower leg is bent, the growth of the limb on the affected side slows down. Accordingly, the curvature of posture is often detected. The process is especially unfavorable if the femur and pelvic bones are affected simultaneously.

Monoosseous pathology has a more favorable course. Depending on the type of disease and severity, the degree of deformation varies. For example, if the lower jaw is affected, the bite may change, a pronounced asymmetry of the face may appear, and if the ribs are involved, the chest is deformed.

Which doctor treats fibrous dysplasia?

Fibrous dysplasia is a complex disease. His treatment is primarily engaged in the surgeon or traumatologist. If tumor processes are detected, it is necessary to involve an oncologist.

Diagnostics

Diagnosis of the disease is not difficult for an experienced radiologist. It is necessary to detect foci in the picture of the limb, resembling cloudy glass or speckled zones, which alternate with areas of dense tissue. In some cases, if the lesion of one bone is clearly visible and it is necessary to exclude the involvement of other parts of the skeleton, densitometry is performed. A CT scan may also be recommended.

Monoosseous pathology can be difficult to diagnose. In this case, dynamic observation is recommended. Also, the patient needs to visit a therapist, oncologist, phthisiatrician. Often, for the differentiation of tuberculous lesions of the bones, it is required to pass tests for tuberculosis.

Treatment

Fibrous dysplasia is treated mainly with the help of surgical techniques. It is recommended to remove the affected bone structures and replace them with grafts. If pathological fractures are found, then the Ilizarov apparatus is applied to the patient.

If the pathology is polyosseous in nature, for example, dysplasia of the upper jaw, bones of the skull and limbs is found, measures are recommended to prevent pathological fractures. The patient is given a massage, prescribed physiotherapy, physiotherapy exercises. Processes are continuously monitored.

Medicines are not used in therapy due to their inefficiency. When drugs are prescribed, they are used mainly to correct comorbidities or relieve symptoms.

Prevention

100% effective methods Prevention of fibrous dysplasia does not exist, since the causes of this disease are unknown. Women who care about the health of their unborn child are advised to:

• refuse to work in hazardous industries;
• stop drinking alcohol and smoking;
• carefully monitor your diet, controlling the intake of useful vitamins, macro- and microelements;
• timely take control of endocrine diseases that can adversely affect the development of the fetus;
• choose the optimal mode of work and rest, so that the impact of stress during pregnancy is minimal.

The prognosis for fibrous dysplasia is generally regarded as positive. The greatest danger is the polyostotic form of the disease, leading to severe deformities and a decrease in the quality of life.

The probability of malignancy is 0.2%, and if the patient is under constant dynamic observation, oncology can be detected in the early stages of its development, which allows taking measures.

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Fibrous dysplasia [FD] (or Braitsev-Lichtenstein disease) is a benign pathological process in which fibrous connective tissue grows in the bones of the skeleton instead of normal bone tissue (in its morphology, FD is close to true tumors, it is referred to as tumor-like processes). The basis of fibrous dysplasia, according to most experts, is an anomaly in the development of osteoblastic mesenchyme, which loses its ability to transform into cartilage and bone tissue and transforms into fibrous connective tissue. The disease begins in childhood, but due to the frequent long-term asymptomatic course, it can be detected at any age. Among the precipitating factors highest value have injuries, local infection, metabolic disorders, toxicosis of the pregnant woman, extraction of teeth, UHF therapy. Activation of the growth of the current process occurs after a biopsy, the use of oral contraceptives, the formation menstrual cycle. One of the likely reasons is the impact of toxic industrial waste on the body of the pregnant woman and the fetus. Based on the fact that the process is often detected in early childhood, it can be assumed that it is congenital (mutation of the gene encoding the α subunit of the G protein on chromosome 20q13.2 - 13.3). PD slowly progresses, and after the onset of puberty it stabilizes, however, the affected bone organ remains structurally and functionally defective for life.

FD most often occurs in childhood and, according to a number of authors, makes up from 2 to 18% of tumor-like bone formations, of which about 20-40% is maxillofacial localization. Most often, the disease is diagnosed at the age of 6-15 years, which corresponds to the period of growth of the facial skeleton, change and growth of teeth, changes in the level of sex hormones. The structure of the disease is dominated by the female sex.

There are mono- and polyostotic forms of FD, as well as Albright's syndrome; according to the nature of the lesion - focal and diffuse. A.A. Kolesov differentiates this pathology into 3 histological forms. [ 1 ] In the first - the main - form, the proliferation of cellular fibrous tissue is noted, against the background of which bone formations are scattered in the form of a few beams; [ 2 ] in the second - proliferating - form, together with the zones of bone trabeculae in certain areas, the cells are densely packed; [ 3 ] in the third - osteoclastic - form, a large number of osteoclasts collected in nodules are found. Monoosseous form - the most common, occurs in 70% of cases. Most often, the ribs and the craniofacial region are affected, while the rest of the skeleton retains a normal structure. Bone lesions may be asymptomatic. The polyosseous form occurs in 30% of cases. The most severe form is Albright's syndrome, which is characterized by a triad of signs: polyostotic form of fibrous dysplasia, premature sexual development (menarche at the age of 3 years) and skin pigmentation (light coffee-colored spots that do not rise above the level of the skin).

The variety of clinical symptoms and the long period of formation of symptoms of the disease makes the diagnosis extremely difficult. The percentage of diagnostic errors in this disease is still quite high, since the clinic of individual localizations of this lesion has not been studied.

Isolation of the FD of the skull into a separate form is very conditional and, most likely, justified from a clinical point of view. With the multiple nature of the lesion of the skeleton, the bones of the skull are involved in the process in 50% of cases. More often the bones of the anterior part of the skull are affected: frontal, sphenoid, maxillary, ethmoid. Less commonly, the parietal and occipital bones are involved in the process. Clinically, this localization of FD is manifested by varying degrees of deformation of the skull, primarily its facial part, as well as symptoms of compression of the surrounding structures. At the same time, it should be noted that gross organic disorders on the part of various departments brain do not occur, since the described changes develop slowly, during the entire period of skeletal formation, which provides a fairly complete compensation. However, in some patients, moderate lethargy, mental retardation, and some decrease in intelligence are still detected. From the age of 25 - 30 years, headaches, dizziness, increased fatigue, angiodistonic syndrome (lability of the pulse and pressure during various tests) may appear.

With damage to the anterior cranial fossa, i.e. fronto-orbital region, the following symptoms may occur: narrowing of the orbit, its displacement from top to bottom and outwards, exophthalmos, hypertelorism (change in the distance between the orbits), oculomotor disorders, defect in binocular vision, thickening of the eyebrow. For complaints about headache in the frontal region, the clinic of the "frontal psyche" can join. With damage to the perpendicular plate of the ethmoid bone and the vomer (nasal septum), there is difficulty in nasal breathing and constant lacrimation due to compression of the lacrimal-nasal canals. In cases of pronounced changes in the bones of the facial skeleton, the shape of the patient's face is distorted and outwardly resembles the concept of "lion's face" (Leontiasis ossea), widely used in various pathological processes. When the middle cranial fossa is involved in the process, atrophy of the optic nerve can develop, as a rule, of a unilateral nature, followed by visual impairment of varying severity. Deformation of the Turkish saddle is possible, but there is no violation of the function of the pituitary gland. With the defeat of the Blumenbach clivus and the lateral parts of the middle cranial fossa, slightly pronounced organic symptoms occur: anisocoria, weakness of the chewing muscles, asymmetry of the nasolabial folds, decreased pharyngeal reflexes, dysarthria, hyperflexia with pathological signs, and sometimes convulsive seizures. When the process spreads to the stony part of the temporal bone, symptoms of damage to the auditory nerve may appear: a decrease or loss of hearing, and pathological sound sensations (ringing, noise) are also possible. Sometimes there is trigeminal neuralgia or hypoesthesia in its zone. When the scales of the occipital bone are affected, head movement is limited. With a significant deformation of the occipital bone, persistent neuralgia of the occipital nerve may occur. However, no dysfunction of the cerebellum is observed. The defeat of the upper jaw may be accompanied by deformation of the face, displacement eyeball up, moderate exophthalmos, overhanging of the upper eyelid, constant lacrimation and often recurrent conjunctivitis, loosening and loss of teeth, changes in bite. With the development of fibrous dysplasia in the lower jaw, its deformation and increase in size of varying severity occur, as a result of which the patient's cheeks are pulled down, dragging the lower eyelids with them, and the lower parts of the sclera are open.

Its transformation into a malignant tumor is important for the clinic and prognosis of FD. Causes of malignant degeneration: not radically performed surgical interventions, the use of X-ray therapy, physiotherapy. Malignancy of fibrous dysplasia occurs in 0.4 - 1% of cases. The appearance of pain against the background of a calm course of fibrous dysplasia without previous trauma is an important signal to the doctor for additional CT diagnostics or biopsy.

Differential diagnosis of the monoosseous form of FD is often difficult, especially when there is only one limited area of ​​rounded lucency on the radiograph, which can be mistaken for an odontogenic cyst or osteoma. The osteoma on the radiograph shows a more intense homogeneous formation with clear contours. FD must be differentiated from osteoblastoclastoma, osteogenic sarcoma, cementoma, eosinophilic granuloma, bone lesions in meningeoma, hyperthyroid dystrophy, deforming ostosis (Paget's disease), osteomyelitis of flat bones, hyperplastic processes of an inflammatory or traumatic nature.

At present, sufficient information has been accumulated in the domestic and foreign literature on the X-ray diagnosis of fibrous dysplasia. Many authors consider the X-ray method to be more accurate than the histological one. X-ray diagnostics makes it possible to detect structural changes in bones even when there is no change in the shape of the bone. With fibrous osteodysplasia of the bones of the facial skeleton, the x-ray picture is quite diverse. The bone increases in volume in all directions, its shape and dimensions change, acquiring the appearance of a “swollen” bone, the cortical layer becomes thinner, the boundaries of the lesion are quite clear. Against the background of a finely reticulated pattern of the bone, many homogeneous cyst-like enlightenments are revealed, separated by rims of coarse trabeculae. The x-ray picture of the lesion of the bone area may be diffuse or limited. In the focal form, well-defined rounded foci of enlightenment in the bone appear, having a clear border in the form of a sclerotic area. The diffuse form is characterized by rarefaction of bone tissue without clear boundaries, the surrounding tissue is porous. Three forms of bone tissue dysplasia were identified radiographically: sclerotic, pseudocystic, and paget-like. CT scan most useful in the evaluation of maxillofacial lesions and allows to identify the degree of involvement of different areas, helping to determine the nature of the surgical intervention. CT allows you to determine not only the boundaries, but also the density of the neoplasm, which can help in differential diagnosis with other pathological conditions, such as osteomyelitis. Fibrous dysplasia is characterized by high density values ​​(70 - 130N). The final diagnosis requires morphological confirmation. Laboratory data in this disease do not play a special role - metabolic processes, as a rule, are not disturbed.

The difference in etiological factors and various clinical manifestations diseases led to the absence of any single tactics for the treatment of FD. At different times, attempts were made to medically correct impaired osteogenesis - calcium preparations, hormones, vitamins, phosphates, etc. were used. However, the experience of their use is small and is based on single observations. There have been attempts to use radiation therapy, but the results were unfavorable. Currently, the main method of treatment is surgery. The intervention technique is discussed by many authors: sparing surgery, radical excision of areas of impaired osteogenesis without or with bone graft implantation. The authors are based on their own isolated observations. The monoosseous form of fibrous dysplasia of the bones of the facial skull should be surgically treated as early as possible and in the amount of complete resection of the pathologically altered bone. Surgical treatment should be carried out in the period of stabilization of the process, that is, in adulthood. Some authors consider only dysfunction of the affected organ to be an indication for surgical intervention. Corrective interventions are performed as in any other tumor-like formations, according to the principles of modern oncology. In this case, a method is chosen that gives the best result in each specific case - it is better to carry out resection (partial or complete) with rapid growth pathological focus, progressive restructuring of bone tissue (according to x-ray indicators), pain, dysfunction, and cosmetic defect. Relapses of the disease, progression or malignancy after surgical treatment, according to some authors, reach 8-10%. Success in treatment does not correct for the size of the lesion. There is not enough data for a comparative description of the methods of surgical treatment. Thus, when choosing surgical method treatment, it is necessary to take into account the type and nature of the neoplasm, localization, age and condition of the patient, as well as the possibility of subsequent restoration of the shape and function of the damaged bone and surrounding tissues.