Fibrous dysplasia of the ENT organs: causes, symptoms, diagnosis, treatment

Tumor-like lesion of bone tissue. A rare pathology in otolaryngology. The basis of the disease is the destruction of bones with their deformation and filling of the bone marrow canal with fibrous tissue. In recent years, an increase in the number of children with dysplastic bone tissue lesions has been noted.

Synonyms

Cystic osteodystrophy, fibrous osteitis, deforming osteodystrophy.

ICD-10 code

M85.0 Fibrous dysplasia.

Epidemiology of fibrous dysplasia of ENT organs

It accounts for about 2% of bone tumors, of which 20% of cases are in the maxillofacial localization. Of the ENT organs, the paranasal sinuses are predominantly affected. Involvement of the temporal bone occurs quite rarely.

Screening

It is extremely difficult due to the very slow development of the fibroplastic process, which causes common inflammatory diseases of the paranasal sinuses and temporal bone in the early stages of development.

Classification of fibrous dysplasia of ENT organs

There are monostotic (70-81.4%) and polystotic (30-60%) lesions. In the polystotic form, the most common symptom is facial asymmetry; functional disorders are less common. There are also proliferative, sclerotic, and cement-forming forms of fibrous dysplasia. The sclerotic type of fibrous dysplasia and the mixed type with a predominance of the sclerotic component are characterized by a favorable prognosis after surgical treatment and the absence of significant progression of the process with age. The proliferative type of dysplasia is characterized by the emergence of a tendency to progressive growth in early childhood, but stabilization of the process in puberty. The greatest difficulties in treatment are caused by cement-forming fibrous dysplasia, which is most prone to recurrence in childhood.

By localization of bone tissue damage, monostotic fibrous osteodystrophy is divided into a widespread form and an isolated focus form (monolocal form), polystotic fibrous osteodystrophy is divided into monoregional, polyregional and disseminated forms. By the nature of bone tissue damage, diffuse, focal and mixed damage are distinguished; by the stage of bone tissue damage - active and stabilized dysplasia.

Causes of fibrous dysplasia of the ENT organs

The etiology of the disease is unknown.

Pathogenesis of fibrous dysplasia of ENT organs

During fibrous dysplasia, two periods are distinguished: the period of progression during the growth and development of the body and the period of stabilization of the pathological condition, typical for adults. The most intensive progression of the pathological process is observed during the period of growth of the child before reaching puberty. The cyclical nature of the disease and stabilization of the lesion after the final development of the child's body are characteristic. The development of dysplasia is usually accompanied by an increase in the volume of the affected part of the skull, which leads to functional disorders of nearby organs. Progression of the disease in an adult indicates the emergence of complications of the primary lesion or its occurrence against the background of a qualitatively different pathological process.

Symptoms of fibrous dysplasia of the ENT organs

It is characterized by a variety of clinical symptoms and a long preclinical period, dependence of the course on age, localization, number of bones involved in the pathological process and the rate of its spread. Despite the fact that fibrous dysplasia is a benign formation in its histological structure, its clinical course is closer to tumors with malignant growth, since it has the ability to quickly destructive growth, compression and dysfunction of nearby organs. In the early period of development, there are very few symptoms characteristic of this disease. Often, one of the first symptoms of this disease is the occurrence of local inflammatory processes (sinusitis, otitis). Gradually, asymmetry and deformation of the facial skeleton, the presence of a slowly increasing dense painless swelling (hyperostatic proliferation) in the affected area become noticeable. The skin in the area of swelling is not inflamed, it is of normal color, thinned, atrophied, shiny; hair on the skin of the tumor-like formation is absent. Malaise, headache, hearing and vision impairment are characteristic. Most patients with craniofacial localization of fibrous dysplasia have a monostotic variant of the lesion, which causes the greatest difficulties in diagnosis.

Bone dysplastic lesions of the temporal bone are characterized by narrowing of the auditory canal due to bone protrusion, mainly of the upper wall, and scanty purulent discharge.

In case of inflammation or exacerbation of the pathological process, pain appears during percussion and palpation of the formation areas.

Diagnosis of fibrous dysplasia of ENT organs

The main diagnostic method is three-dimensional CT, which allows determining the prevalence and localization of the process and, accordingly, the scope of surgical intervention, as well as fibroendoscopy.

In the anamnesis, they try to find out the cause of the disease, early clinical manifestations, the scope and nature of home examination and treatment, concomitant diseases of internal organs and ENT organs.

Physical examination

Palpation, percussion of the tumor-like formation: determination of hearing and vision acuity.

Laboratory research

Histological examination reveals spongy and compact bone tissue. In compact bone, there is uneven ossification; the tumor consists of round and oval, large and small cavities (cysts) of varying sizes, containing a brownish (chocolate-colored) mushy gelatinous mass; some of them may be filled with purulent contents. The outer bone walls of the tumor and the bone bridges of the cells are distinguished by a pronounced density like ivory. In spongy bone, there is a sharp thinning of the bone beams, expansion of the bone marrow spaces filled with finely fibrous tissue rich in fibroplastic cells and adipocytes. Among the fibrous tissue, there are foci of forming primitive bone tissue.

Bleeding during surgery is usually minor.

Instrumental research

Radiologically, formations of a heterogeneous structure with cysts of various sizes are determined (the heterogeneity is due to multiple areas of enlightenment with clear contours). The outer walls of the tumor and bone bridges are of a very dense consistency (like ivory).

Differential diagnostics of fibrous dysplasia of ENT organs

It is performed with benign and malignant neoplasms of the maxillofacial region, hyperplastic processes of inflammatory and traumatic nature, Langerhans cell histiocytosis, inflammatory diseases of the paranasal sinuses and temporal bone.

Indications for consultation with other specialists

Taking into account the damage to adjacent anatomical structures, consultation with an ophthalmologist and audiologist is recommended.

Treatment of fibrous dysplasia of ENT organs

Treatment goals

Elimination of deformation of the facial skeleton and skull with the most gentle version of surgical intervention in children - taking into account the ongoing growth and development of the bones of the skull.

Indications for hospitalization

Progressive deformation of the face and skull, signs of suppuration of fibrous dysplasia.

Non-drug treatment

They don't.

Medicinal cookies

Vitamins, general strengthening and immunocorrective treatment.

Surgical treatment

The leading role is given to surgical treatment of the disease. In childhood, the most gentle nature and volume of surgical intervention is more justified: excision of fibrous dysplasia within healthy tissue is possible in a smaller volume - taking into account the ongoing growth and formation of the bones of the facial skeleton and skull. In adults, total resection of the affected bone is mainly performed with subsequent plastic reconstruction.

Indications for surgical intervention include the presence of deformation, functional impairments, and a tendency toward progressive growth without signs of maturation of dysplastic tissue. When determining the tactics of patient management and determining the scope of surgical intervention, the morphological type of fibrous dysplasia is taken into account.

During the period of the child's growth, in the absence of intensive growth and complications, they try to refrain from surgical treatment if possible. In the presence of a pronounced change in the face, causing its disfigurement, suppuration from a violation of the trophism of the tissues of fibrous dysplasia, especially during the period of a stabilized pathological condition, surgical intervention is indicated - removal of the tumor-like formation and restoration of the shape of the face. This also serves as prevention of suppuration of the pathological process. The tumor cavity is opened with a chisel and pathological tissue is removed to the borders of healthy bone.

Further management

Long-term observation by an otolaryngologist for timely diagnosis of signs of inflammation and progression of osteodysplastic growth,

Forecast

Early radical surgical intervention provides a guarantee of success only if the morphological structure of the process is taken into account.

Prevention of fibrous dysplasia of ENT organs

Difficult because the etiology is unknown.

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