Dysplasia of the skull bones. Fibrous dysplasia of bone. Forms of fibrous dysplasia

Patient D. Date of birth: 05/18/2005 (11 years old).

Anamnes morbi : 05/18/2005 has been ill since September 2014, when facial asymmetry first appeared - an increase in the middle zone of the face on the left. In dynamics, the deformation increased moderately. We contacted the State Budgetary Institution “Scientific Center for Special Medical Assistance to Children of the Department of Health”, where in February 2014 he underwent inpatient treatment - partial resection of the upper jaw on the left; according to histological examination, fibrous dysplasia of the upper jaw was diagnosed. Subsequently, asymmetry and deformation of the middle zone of the face on the left remained without dynamics. On January 20, 2017, he was hospitalized in the Department of Maxillary Surgery and Dentistry of the State Budgetary Healthcare Institution “Special Medical Center for Special Medical Assistance to Children of the Department of Health”.

The condition during hospitalization was of moderate severity; stable. No pathology of organs and systems was identified.

After a consultation of doctors, it was decided to perform planned surgical treatment in the amount of partial resection of the body of the zygomatic bone on the left, the alveolar process of the upper jaw on the left with revision of the maxillary sinus on the left using a multifunctional argon plasma system Plasmajet.

Rice. 1. Appearance of the patient in frontal and semi-axial projections

CT scan of the skull dated January 24, 2017 – thickening and deformation of the greater wing of the sphenoid bone on the left, the zygomatic bone and the upper jaw with thinning of the cortical plates remain. The superior orbital fissure and foramen rotundum on the left are narrowed. The lateral wall of the left orbit bulges, deforming the muscle cone. The left maxillary and sphenoid sinuses are filled with pathological formations. No areas of pathological accumulation of the contrast agent were identified.

Rice. 2. CT scan in axial and frontal projections. The area of ​​fibrous dysplasia is highlighted in red.

Conclusion: CT picture of fibrous dysplasia of the sphenoid, zygomatic bones and upper jaw on the left.

02/01/17 a team of doctors headed by prof. Zarichansky V.A. performed the operation.

After treating the oral cavity with an aqueous solution of chlorhexidine bigluconate, m\a and hydropreparation using a 0.5% solution of lidocaine - 10.0 ml, an incision was made in the mucous membrane along the transitional fold of the upper fornix of the vestibule of the oral cavity on the right in the area of ​​the postoperative scar, about 3. 5 cm. The body of the upper jaw is sequestered.

Rice. 3. The upper jaw is skeletonized. Tumor removal

The bone is changed, hyperplastic, fills the entire cavity of the maxillary sinus, the body and alveolar process of the upper jaw on the left, extending to the lateral wall of the nose, the hard palate and extending to the body of the zygomatic bone. The altered bone tissue was removed in the area of ​​the alveolar process on the left, the body of the upper jaw (projection of the maxillary sinus) on the left, and part of the body of the zygomatic bone on the left. Hemostasis during surgery using wax, a multifunctional argon plasma system Plasmajet in order to reduce bleeding and radicality of the operation, as well as the diathermocoagulation method. For orthodontic reasons, tooth 6.3 was removed. The removed parts of the altered bone and fragments of scarred soft tissue are sent for histological examination.

The operation was carried out under the guidance of the director of the center, prof. Prityko A.G., operating surgeon prof. Zarichansky V.A., assistants: Molodtsova N.A. and Saidova G.B.

Among various diseases and malformations leading to deformations of the facial skull, fibrous dysplasia, which was identified as an independent nosological form only by 1940, should be noted. The essence of this disease comes down to the development of foci of bone tissue damage, in which it is replaced by vascular-poor cellular fibrous and fibrous tissue.

Along with this, new formation of bone structures is also noted; however, this process, as a rule, is imperfect and is characterized by the irregularity of the newly formed bone tissue, the diversity of its structure, the appearance of defective structures, and pronounced resorptive changes. The affected areas are clearly demarcated from normal bone tissue and can be located in any area of ​​the skeleton.

Damage to the bones of the facial and cerebral parts of the skull is described as one of the types of fibrous dysplasia, called “bone leontiasis” or “hemicraniosis”. The first name is due to extensive bone and fibrous growths that create a characteristic deformation of the skull, in which the patient’s face resembles the head of a lion. The term "hemicraniosis" indicates the frequency of unilateral lesions of the skull. It should be noted that the term “bone leontiasis” does not reflect the essence of the process, but only indicates external manifestations of deformation, which can be the same type for diseases of different pathoanatomical nature. However, most often this deformation accompanies fibrous dysplasia of the skull bones.

Despite the extensive literature on fibrous dysplasia, the true cause of this disease still remains unknown. Only one form of dysplasia - Albright - is often combined with hormonal disorders. For other types of dysplasia, no significant endocrine changes have been described. With fibrous dysplasia, there are no biochemical or hematological changes or pain.

Fibrous dysplasia occurs among representatives of any continent, but the maximum frequency of lesions of the facial skull is noted in West Africa. In most cases, the disease begins in childhood and primarily affects females. Slow progression of the lesion usually occurs until the end of puberty, after which the process usually stabilizes. Cases of malignancy of bone lesions are extremely rare and unreliable, but pathological fractures do occur, especially in the bones of the extremities, and usually it is the “spontaneous” injury that first reveals the dysplastic bone process. Surgical interventions, especially before the end of puberty, are ineffective, as they lead to a steady recurrence of the lesion.

Based on radiological manifestations, dysplasia in the bones of the skull can be divided into several types:

a) sclerotic;

b) cyst-like,

c) paget-like.

Sclerotic type

Characterized by the formation of areas of intensive restructuring of bone tissue, its compaction, most often in the area of ​​the base of the skull, frontal and maxillary bones, and nasal bones. The paranasal sinuses in the area of ​​bone growths completely lose their airiness and are replaced by dense, structureless bone tissue. If the bones that form the orbit or nasal cavity are affected, deformation and narrowing of these cavities, exophthalmos, impaired nasal breathing, and neurological symptoms occur due to compression of the nerve trunks in the narrowed canals. Lesions are often unilateral or asymmetrical. The process usually does not spread through the sutures of the skull roof.

Cyst-like type

Most often it develops in the mandibular bone. “Cysts” can be single or multiple, often have a wavy contour and a clear cortical rim, an almost regular round shape.

Within clearing zones, small punctate foci of calcification or islands of structureless bone tissue may occur. The cortical layer along the lower edge of the jaw body becomes loose, often expands from the inside, but is not interrupted anywhere. Osteoporosis and periosteal reaction are absent.

Areas of compacted bone tissue alternate with zones of clearing, but without sharp thickening of the cortical layer. In the two cases we have, the changes were localized in the occipital and frontal bones and were combined in both patients with sclerotic restructuring of the bones of the base of the skull and individual facial bones.

It should be noted that in half of the patients we observed, along with changes in bone tissue in the same parts of the jaws, various defects in the formation of teeth or accumulations of lumps of cement could be noted, i.e. osteocementodysplasia occurs.

In the differential diagnosis of fibrous dysplasia, one must first take into account Paget's disease, which usually affects older age groups and predominantly men. Changes most often occur in the skull, but they are almost never isolated, and a characteristic restructuring of the bone structure is detected simultaneously in the vertebrae, pelvic bones and lower extremities.

Deformation of the skull in Paget's disease is expressed in an increase in the size of the cerebral part of the skull, caused by a significant thickening of the bones of the entire vault. The cranial cavity may even shrink. The normal structure of the integumentary bones disappears, the outer compact plate sharply thickens, becomes rough, and instead of the trabecular diploe pattern, small multiple rounded areas of bone tissue compaction appear, alternating with zones of clearing. The bones of the facial skull are usually not changed, and the area of ​​the base of the skull is sclerotic. The enlarged cerebral part of the skull seems to hang over the facial part. The anterior cranial fossa protrudes upward, and the sella turcica flattens. The paranasal sinuses have an unusually large volume. In addition to deformation of the cranial cavity, the pelvis and vertebral bodies are also sharply deformed.

Fibrous dysplasia is a bone disease based on a tumor-like process associated with improper development of osteogenic mesenchyme. In this case, the process of replacing bone tissue with fibrous tissue occurs, as a result of which bone deformation occurs. The reasons for the development of this pathology are not clear enough. Depending on the distribution of the lesion, monoostotic (when one bone is involved in the process) and polyostotic (when several bones are affected) forms of fibrous dysplasia are distinguished.

In the available literature, we were unable to find information about fibrous dysplasia of the skull base, and therefore we consider it advisable to present our own observation of a patient with polyostotic fibrous dysplasia of the skull base, in whom we performed an operation using our own technique to remove the lesion.

Patient N., 35 years old, was admitted to the department on March 14, 1991 with complaints of constant headache, protrusion of the left eyeball and double vision. The disease began in early 1989, when the patient noticed a protrusion of the left eyeball. Later this was accompanied by headache and double vision. When visiting a doctor, the patient was diagnosed with glaucoma and prescribed symptomatic treatment, which did not lead to an improvement in her condition. A CT scan revealed a tumor at the base of the skull. The patient was referred to our department to decide on the possibility of surgical intervention.

Upon admission, the patient's condition was satisfactory; left-sided exophthalmos was noted. No pathological changes were found in the internal and ENT organs. Blood and urine test results were without pathological changes.

The ophthalmologist's conclusion: left-sided exophthalmos, initial symptoms of stagnation of the optic nerve nipple. Vision is satisfactory (Vis.OD = 1.0; OS = 0.9). The mobility of the eyeballs is not limited.

Computed tomograms show a compact compaction, characteristic of dysplastic bone disease, localized mainly in the body of the sphenoid bone on the left (Fig. 1, a. Foci of “swelling” of the sphenoid bone are visible, due to which its body acquires a rounded shape. The lesion covers the upper, the anterior and lateral surfaces of the greater, as well as the lower surface of the lesser wing of the sphenoid bone. Due to the deformation of the posterior parts of the upper and outer walls of the left orbit, the superior orbital fissure is narrowed. Compression of the optic nerve and anterior displacement of the left eyeball are noted (Fig. 1, b).

On April 25, 1991, an operation was performed to remove a focus of fibrous dysplasia of the skull base using an approach through the infratemporal fossa on the left. Under endotracheal anesthesia, an arcuate skin incision was made, starting from the anterior edge of the left temporal region and ending at the level of the auricle. After dissection of the subcutaneous fat layer, the skin flap was separated and displaced until the temporo-parotid region was exposed. Next, the zygomatic arch on both sides is dissected obliquely, after which the temporal muscle is separated from the bone (later the muscle is used to obliterate the postoperative cavity). After downward displacement of the mobilized temporal muscle and the severed zygomatic arch (together with the attached masseter muscle), the ligaments of the temporomandibular joint were dissected. Then, using a Gosse retractor inserted into the surgical wound (one hook of the retractor is inserted into the articular fossa), the head of the mandible is shifted downwards. This made it possible to expand the surgical wound, separate the soft tissue from the base of the skull and expose the infratemporal fossa. The isolated middle artery of the dura mater is coagulated and dissected near the foramen spinosum. Starting from the lateral surface of the greater wing of the sphenoid bone, under the control of an operating microscope, using a bur and electric suction, the infratemporal crest and the zygomatic edge of the temporal surface of the greater wing of the sphenoid bone were removed. The bones were resected until the surface of the dura mater of the temporal lobe of the brain was exposed. At this stage of the operation, the infratemporal fossa was reached, on the upper wall of which (at the level of the body of the sphenoid bone) there was a whitish-gray focus of pathological tissue. The visible part of the pathological tissue reached the anterior part of the bottom of the temporal fossa (the lateral surface of the large wing of the sphenoid bone). Using a bur and surgical spoons (under the control of an operating microscope), foci of pathological tissue were removed along with the affected areas of the base of the skull and bones of the facial skeleton. It was established that the upper, anterior and lateral surfaces of the greater wing, as well as the lower surface of the lesser wing of the sphenoid bone were involved in the pathological process. After hemostasis, the postoperative cavity was obliterated with the temporal muscle, which also filled the defects that arose after surgery on the base of the skull, the posterior parts of the upper and outer walls of the orbit. Then the head of the lower jaw is returned to the articular fossa, and the zygomatic arch is fixed with wire in its original place. At the final stage of the operation, the surgical wound was drained and sutures were placed on the skin.

Histopathological examination revealed fibrous connective tissue replacing bone marrow in the specimen. In some of its areas, poorly calcified bone beams are identified, forming spongy bone of varying maturity. In some places, the fibrous tissue consists of chaotically located bundles of mature collagen fibers and spindle cells. Conclusion: fibrous dysplasia.

The patient was discharged from the hospital in good condition on the 31st day after surgery. Exophthalmos and diplopia disappeared. When examined 6 and 12 months after surgery, the patient had no complaints. Computed tomograms showed no signs of disease relapse (Fig. 2).

Thus, this observation indicates the possibility of developing polyostotic fibrous dysplasia at the base of the skull, the recognition of which is difficult. Surgical intervention is the optimal treatment method for patients with this pathology. Radical intervention can be achieved through surgery (according to the author) using an approach through the infratemporal fossa. CT allows you to identify pathological tissue in a given pathology, determine the boundaries of the spread of the lesion and judge the effectiveness of the treatment.

Literature

1. Rzaev R.M. A method for removing a tumor at the base of the skull. Patent 980024 dated November 9, 1992. Official Bulletin of the Patent and Licensing Committee for Science and Technology of the Azerbaijan Republic. Baku 1998; 37-38.

2. Strukov A.I., Serov V.V. Pathological anatomy: Textbook. Ed. 3rd, revised and additional M: Medicine 1993; 688.

The term fibrous dysplasia usually means a violation of the structure of bone tissue, when normal full-fledged tissue of tubular or flat bones degenerates into connective tissue, which includes bone partitions - trabeculae.

The pathological process of bone tissue belongs to the category of tumor diseases. Fibrous dysplasia is local in nature and can spread to large areas of bone. A single bone or several are involved in the pathogenic process. The exact causes of the disease have not been established.

Fibrous dysplasia refers to tumor-like processes, having no relation to true tumor neoplasms. The development of degeneration into connective tissue occurs due to disturbances in the formation of mesenchymal tissue, which is responsible for the normal formation of bone tissue. The first symptoms occur in children already in early childhood. In the medical literature there are descriptions of exceptional cases when fibrous dysplasia, showing a monofocal type of course, was first diagnosed in elderly people.

The female part of the population is more susceptible to the disease compared to men. Sometimes there have been cases where the fibrous tumor was transformed into a benign tumor neoplasm, but malignancy of the process is extremely rare.

Fibrous dysplasia was first introduced into the medical literature a hundred years ago. At the end of the twenties of the last century, a doctor from Russia Braitsov published a scientific report on the signs and symptoms of fibrous degeneration of bone tissue from the point of view of clinic, radiology and microscopic histological picture.

Ten years later, physician Albright described a similar multifocal process. Multiple bone lesions are described in combination with endocrinopathies of all kinds and characteristic changes in the skin. At the same time, another scientist, Albrecht, highlighted in detail the issue of the connection between the multifocal process in bone tissue and disorders of puberty and hormonal maturation. A number of scientists have described monofocal disorders, coming to different conclusions about the etiological nature. Nowadays, fibrous dysplasia is found under the names of many authors.

Types of fibrous dysplasia

In the literature and clinical practice, a classification has been adopted consisting of two main forms - monoostotic and polyostotic. In the first case, there is damage to bone tissue in a single area, the second type of disease affects a number of bones, anatomically related to one side of the body.

The multifocal form usually affects young children and is often combined with endocrinopathies and cutaneous melanosis. The type of disease that affects a single bone can develop at any age and does not entail concomitant endocrinopathies and hyperpigmentation.

In Russian oncological and orthopedic practice, it is customary to use a classification whose author is named after Zatsepin. There are subtypes of the disease:

1. Intraosseous dysplasia is polyostotic and monoosseous in nature. Microscopy of bone tissue reveals focal formations of fibrous connective tissue. Sometimes there are cases of fibrous degeneration of the entire bone body. At the same time, there are no bone deformations, the structure of the superficial bone layer is not affected.
2. Total damage. All areas of the bone are affected, from the cortical layer to the bone marrow canal. Gradually, due to fibrous degeneration, bone deformations develop, leading to frequent fractures. Often the polyostotic process affects the bodies of the femoral tibia and fibula of the leg.
3. Tumor type of degeneration. It is always accompanied by proliferation of fibrous tissue, in some cases reaching colossal sizes. In practice it occurs in isolated cases.
4. Albright's syndrome. The clinical form is often diagnosed in children and combines clinical symptoms: bone dysplasia and endocrine disorders, disorders of the pubertal period, disproportionate development of the hips and lower legs in a child, pigmentation disorders of the skin. There is a pronounced deformation of the pelvic ring and iliac region, femur and fibula bones of the leg. Disturbances in various systems in children are progressive and rapid.
5. Fibrous-cartilaginous dysplasia often degenerates into a malignant neoplasm.
6. Calcifying clinical form. Often the disease affects the body of the fibula and tibia of the leg.

Clinic of the disease

Severe deformations are usually not observed with bone fibrosis. Multifocal lesions are more common in children. Damage to bone tissue is accompanied by disturbances in the activity of the endocrine glands, heart and blood vessels. Symptoms of this form of the disease vary widely. Often children have complaints of pain in the femoral area, which are not intense, and there is a sign of gradual deformation of the bones. In some cases, the disease is detected only after the formation of pathological fractures of the femur or fibula.

The polyostotic type of formation of the disease usually affects the tissue of tubular hollow bones - the femur, tibia, and ulna. Damage to flat areas of the bone is represented by processes in the ilium, skull, scapular bones and bone plates of the rib. Quite often in clinical practice, a characteristic sign is the involvement of the feet or hands, although the wrist is not affected.

The degree of intensity of the lesion directly depends on the site of localization of the pathological process. If the degeneration involves the bony skeleton of the upper limb, it is usually possible to clinically detect only a club-type expansion. When the phalanges of the fingers are affected, they take on a shortened appearance.

The body of the femur and tibia, large and small, bears a significant load, leading to their pronounced curvature and the development of characteristic deformities.

Shortening of the femur is often observed. The pronounced curvature gives the bone the shape of a staff or boomerang. Often the greater trochanter of the femur moves upward and reaches the wing of the ilium. Lameness develops due to deformation of the surgical femoral neck. The shortening of the limb is significant and requires surgical treatment.

If the fibula is damaged, there are no signs of limb deformation. When the tibia is damaged, the shin becomes saber-shaped and stops growing in length. Compared to the development of a pathological process in the femur, the shortening is not so significant, treatment is simpler and more effective.

With the degeneration of the fibrous nature of the body of the ilium, a violation of the structure and functions of the pelvic ring in children develops. Posture disorders develop in the form of kyphosis or scoliosis. If the process in children simultaneously affects the areas of the femur and pelvic bones, spine or ribs, this leads to a greater displacement of the central axis of the body. The load on the spinal area increases more, increases symptoms and requires treatment to be adjusted accordingly.

When a single bone is affected, the clinical picture and prognosis of the disease are considered favorable. Usually the process is limited only by damage to bone tissue and the absence of concomitant disorders of the skin and endocrine glands. The lesion is both widespread and intraosseous, which affects the clinical manifestations. With increased physical activity, children complain of pain in the legs and lameness. In severe cases, there is a risk of pathological fracture, which is treated with the help of an orthopedic device.

Diagnostic research methods

The diagnosis is made based on the following indicators:

• Characteristic complaints and anamnestic data.
• Clinical symptoms.
• X-ray data.
• Consultation with specialized specialists – endocrinologist, cardiologist.

At the first stages of the formation of a pathological process in bone tissue, an area resembling ground glass is revealed on radiographs. As the disease progresses, the bone tissue acquires a characteristic “holey” appearance. There is an alternation of areas of compaction and clearing in the bone tissue. An x-ray or tomogram clearly shows bone deformation of the limbs or flat bones of the skull and chest. If a unique lesion is detected, a thorough examination is required to exclude the possibility of the development of multiple lesions and to prescribe effective treatment.

The initial stages of the pathological process in bone tissue, especially those of a monoosseous nature, may not show clinical manifestations and be an accidental radiological finding when conducting research for completely different reasons. To clarify the diagnosis, it is advisable to conduct densitometry and computed tomography, which can detect abnormalities in the structure of bone tissue.

If the clinical picture of the disease is not too pronounced, long-term clinical observation may be required, especially for young children and adolescents during the maturation period.

Therapeutic approaches

The main treatment for fibrous dysplasia is predominantly surgical. The surgical operation consists of the following: complete excision of the transformed area of ​​bone tissue is performed and the resulting defect is replaced with a bone tissue graft. If a pathological fracture of the bones of the lower limb is detected, treatment is performed using the Ilizarov apparatus. The device is a system of knitting needles and rings made of medical steel. With the help of the device, the bone skeleton is stretched and the normal length and functions of the limb are restored.

The prognosis for life and health with bone dysplasia is considered relatively favorable. If appropriate treatment is not carried out and the operation is cancelled, this will lead to severe deformations of the skeletal system and complete immobilization. Sometimes there are cases when fibrous bone dysplasia transforms into a benign tumor, which forms at the site of the lesion. Several cases of malignancy have been described. Treatment is carried out by an oncologist.

If a child suffering from a multifocal form of the disease has endocrine or cardiovascular disorders, it is necessary that treatment be carried out by a specialist of the appropriate profile in combination with an orthopedist.

Unfortunately, it has not yet been possible to accurately determine the reasons for the changes. Doctors believe that it’s all about 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 forming;
• pathologies of the ligamentous-muscular system, due to which the area of ​​the tibia, femur, and jaws often suffer;
• the presence of a predisposition fixed genetically;
• improper nutrition of a woman while carrying a child (neglect of fruits and vegetables);
• the effect of environmental factors and bad habits;
• use of certain medications during pregnancy;
• severe toxicosis;
• oligohydramnios 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 may be diagnosed, but only on the left or only on the right.

There is another classification, which distinguishes:

• diplasia of the intraosseous type, in which the patient rarely experiences severe bone deformations, and upon examination there are foci of fibrosis in one or more bones;
• total type, in which the entire bone is affected, and the patient exhibits severe deformations of the bone skeleton;
• Albright's syndrome is 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 the pathology are very diverse and depend on the affected joint. If the hip bones are affected, the patient may complain of mild pain, and the deformation will occur gradually. Sometimes the diagnosis is made only after a pathological fracture has formed.

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

The lower limbs are always curved. This happens due to the fact that they are forced to support their body weight. 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. At the same time, the patient begins to limp and complain of pain in the joint.

Fibrous dysplasia of the knee joint or tibia can cause a person to have a bowed lower leg and slower growth of the limb on the affected side. Accordingly, postural curvature is often detected. The process is especially unfavorable if the femur and pelvic bones are affected simultaneously.

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

Which doctor treats fibrous dysplasia?

Fibrous dysplasia is a complex disease. Its treatment is primarily carried out by a 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 image of the limb that resemble cloudy glass or speckled areas, which alternate with areas of dense tissue. In some cases, if damage to one bone is clearly visible and it is necessary to exclude involvement of other parts of the skeleton, densitometry is performed. A CT scan may also be recommended.

Monostotic pathology can be difficult to diagnose. In this case, dynamic monitoring is recommended. The patient also needs to visit a therapist, oncologist, or phthisiatrician. Often, to differentiate tuberculous bone lesions, it is necessary to take tests for tuberculosis.

Treatment

Fibrous dysplasia is treated mainly using surgical techniques. It is recommended to remove the affected bone structures and replace them with grafts. If pathological fractures are detected, the patient is given an Ilizarov apparatus.

If the pathology is polyostotic in nature, for example, dysplasia of the upper jaw, skull bones and limbs is detected, measures to prevent pathological fractures are recommended. The patient is given a massage, physiotherapy and physical therapy are prescribed. Dynamic monitoring of processes is constantly carried out.

Medicines are not used in therapy due to their ineffectiveness. If drugs are prescribed, they are used mainly to correct concomitant diseases or relieve symptoms.

Prevention

There are no 100% effective methods for preventing fibrous dysplasia, 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 beneficial vitamins, macro- and microelements into the body;
• promptly take control of endocrine diseases that can negatively affect the development of the fetus;
• choose the optimal work and rest schedule so that the impact of stress during pregnancy is minimal.

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

The probability of malignancy is 0.2%, and if the patient is under constant dynamic monitoring, oncology can be detected in the early stages of its development, which makes it possible to take action.

Useful video about fibromuscular dysplasia

There are no similar articles.