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

Tumor damage to bone tissue. A rare pathology in otorhinolaryngology. At the heart of the disease is the destruction of bones with their deformation and the filling of the osseous canal with fibrous tissue. In recent years, there has been an increase in the number of children with dysplastic lesions of bone tissue.

Synonyms

Cystic osteodystrophy, fibrous osteitis, deforming osteodystrophy.

ICD-10 code

M85.0 Fibrous dysplasia.

Epidemiology of fibrotic dysplasia of ENT organs

It accounts for about 2% of bone tumors, of which 20% of cases occur in maxillofacial localization. Of the ENT organs, the paranasal sinuses are mainly affected. The involvement of the temporal bone is rare enough

Screening

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

Classification of fibrotic dysplasia of ENT organs

Isolate mono-osseous (70-81.4%) and polyossal (30 60%) lesions. In polyosseous form, the most common symptom is the asymmetry of the facial skeleton, functional disorders are less common. Also, proliferative, sclerotic and cement-forming forms of fibrous dysplasia are isolated. For a sclerotic type of fibrous dysplasia and a mixed type with predominance of the sclerotic component, a favorable prognosis after surgical treatment and the absence of pronounced progression of the process with age are characteristic. In the proliferative type of dysplasia, the appearance in early childhood of a propensity for progressive growth, but the stabilization of the process in the pubertal period is characteristic. The greatest complexity in the treatment causes cement-forming fibrous dysplasia, which is most prone to recurrence in childhood.

By localization of bone tissue damage, mono-osseous fibrotic osteodystrophy is divided into a common form and shape of an isolated focus (monolocal form), polyosseous fibrous ostodystrophy under is divided into mono-regional, poly-regional and disseminated forms. By the nature of the defeat of bone tissue, diffuse, focal and mixed lesions are distinguished; at the stage of lesion of bone tissue - active and stabilized dysplasia.

Causes of fibrotic dysplasia of ENT organs

The etiology of the disease is unknown.

Pathogenesis of fibrotic dysplasia of ENT organs

In the course of fibrous dysplasia, two periods are distinguished: the period of progression during the period of growth and development of the organism and the period of stabilization of the pathological condition typical for adults. The most intensive progression of the pathological process is observed in the period of the child's growth until puberty. The cyclic nature of the course of the disease and the stabilization of the lesion after the final development of the child's organism are characteristic. The development of dysplasia, as a rule, is accompanied by an increase in the volume of the affected skull, which leads to functional abnormalities on the part of the nearby organon. Progression of the disease in an adult indicates the appearance of complications of the primary lesion or its appearance against a qualitatively different pathological process.

Symptoms of fibrotic dysplasia of ENT organs

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

For bone-dysplastic lesion of the temporal bone is characterized by a narrowing of the auditory passage due to the bony convexity of the predominantly upper wall, scant purulent discharge.

When the inflammation or exacerbation of the pathological process appears soreness with percussion and palpation of the sites of education.

Diagnosis of fibrotic dysplasia of ENT organs

The main method of diagnosis is a three-dimensional CT, which allows to determine the prevalence and localization of the process and, accordingly, the amount of surgical intervention, as well as fibroendoscopy.

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

Physical examination

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

Laboratory research

At a histological examination, the bone tissue of a spongy and compact structure is determined. In compact bone - uneven ossification; the tumor consists of different sizes of round and oval, large and small cavities (cysts), containing a brownish (chocolate-colored) pulp-like gelatinous mass; some of them can be filled with purulent contents. The external bone walls of the tumor and the bony crosspieces of the cells differ in pronounced density of the ivory type. In the spongy bone - a sharp thinning of the bony beams, the expansion of bone marrow spaces filled with finely fibrous tissue, rich in cells of the fibroplastic series, adipocytes. Among fibrous tissue - foci of forming primitive bone tissue

Bleeding during surgery is usually small.

Instrumental research

Radiographically, the formation of an inhomogeneous structure with cysts of different sizes (heterogeneity is due to multiple areas of enlightenment with clear contours). External walls of the tumor and bone bridges are very dense in consistency (such as ivory).

Differential diagnosis of fibrotic dysplasia of ENT organs

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

Indications for consultation of other specialists

Taking into account the defeat of neighboring anatomical formations, consultation of an ophthalmologist and an audiologist is recommended.

Treatment of fibrotic dysplasia of ENT organs

Objectives of treatment

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

Indications for hospitalization

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

Non-drug treatment

Do not spend.

Medication-based baking

Vitamins, restorative and immunocorrective treatment.

Surgery

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

Indication for surgical intervention is the presence of deformity, functional disorders and a tendency to progressive growth without signs of maturation of dysplastic tissue. In determining the tactics of patient management and determining the extent of surgical intervention, the morphological type of fibrous dysplasia is taken into account.

In the period of child's growth, in the absence of intensive growth and complications, try to refrain from surgical treatment whenever possible. In the presence of a pronounced change in the face causing its disfigurement, suppuration from trophic tissue disorder of fibrous dysplasia especially during the period of stabilized pathological condition, surgical intervention is shown - removal of tumor formation and restoration of the face shape. This also serves to prevent the suppuration of the pathological process. The chisel opens the tumor cavity and removes the pathological tissue to the boundaries of the healthy bone.

Further management

Long-term follow-up by an otolaryngologist for the timely diagnosis of inflammation and progression of osteodysplastic growth,

Forecast

Early radical surgery provides a guarantee of success only when the morphological structure of the process is taken into account.

Prevention of fibrotic dysplasia of ENT organs

Difficult, because etiology is unknown.

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