Scleroma of the larynx: causes, symptoms, diagnosis, treatment

Laryngeal scleroma is a chronic specific inflammatory process in the mucous membrane of the airways with predominant localization in the nasal cavity and larynx (according to international statistics, 60% in the nasal cavity and 39% in the larynx). Simultaneous lesions of the nose and larynx are often observed. In most cases, the infection debuts in the nasal cavity (rhinoscleroma), but there are also frequent cases of primary laryngeal lesions, which are clinically much more significant, since the resulting scleroma infiltrates invariably end in laryngeal stenosis of varying degrees, up to asphyxia.

Scleroma is common throughout the world, but there are regions where the incidence of scleroma is endemic (Belarus, Ukraine, Poland, Czechoslovakia, certain regions of Serbia, Montenegro, Romania, Switzerland, Indonesia, Central American countries. Small endemic foci are located in Austria, Spain, Asia, Africa).

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Cause of laryngeal scleroma

The pathogenic agent is an encapsulated bacterium similar to Friedlander's bacillus or the microorganism isolated by Abel-Levenberg in patients with ozena. This bacterium was isolated in 1882 by V. Frisch from scleroma infiltrates, from which it can be cultured. Less often, Frisch's bacillus is found in mucous membrane secretions. Scleroma is a practically non-contagious disease, and the microorganism becomes pathogenic only under certain conditions. It is believed that a humid climate, marshy and wooded areas, lack of insolation, and rural living conditions contribute to infection. Females are more often affected. In approximately 5% of cases, children under 15 years of age become ill with scleroma.

Pathological anatomy. Laryngeal scleroma begins with the formation of dense infiltrates in the submucosal layer, consisting of small rounded cells and plasma cells, as well as a large number of spindle-shaped cells and fibroblasts, which complete the formation of the scleroma focus, turning it into a dense tumor. The columnar epithelium located above the infiltrate is transformed into a multilayered squamous keratinized epithelium. The difference between scleroma and other specific diseases of the upper respiratory tract is that the changes in the mucous membrane that occur with it do not ulcerate. As for the scleroma infiltrate, it contains vacuolated foamy cells typical of scleroma, described by Mikulicz. These cells contain small hyaline inclusions (Russel bodies) and clusters of scleroma bacteria, which are often found between Mikulicz cells. Scleromatous lesions evolve over a number of years and then undergo scarring (without disintegration), which leads to the formation of stenotic scars of the larynx, impaired breathing and voice formation.

Symptoms of laryngeal scleroma

The disease begins gradually, manifesting itself in the debut with signs of banal catarrhal laryngitis, then passing into the "dry phase". At the same time, similar phenomena are observed in the nasal cavity. A feature of scleroma foci is their occurrence in narrow places of the upper respiratory tract. Since scleroma infiltrates are localized mainly in the subglottic space, the most pronounced and early sign of laryngeal scleroma is breathing problems, and then, as the inflammation foci spread to the vocal apparatus, dysphonia joins in, progressing to complete aphonia.

Laryngoscopy reveals pale pink infiltrates; in places where the scarring process begins, the infiltrates acquire a whitish tint and become dense to the touch. Infiltrates are usually located symmetrically under the vocal folds, spreading over time to the entire circumference of the larynx. Scleromatous infiltrates have the property of creeping spread both upward, into the area of the vocal folds, and downward, covering the trachea, and sometimes the main bronchi. Much less often, the process begins in the supraglottic space: infiltrates are formed on the laryngeal surface of the epiglottis, on the vestibular and aryepiglottic folds. Sclerosis of infiltrates leads to deformation of the anatomical structures on which they arose. Thus, the epiglottis decreases, shrinks and moves towards the traction of the scar tissue - latral or into the lumen of the vestibule of the larynx. Usually, in the vestibular part of the larynx, in addition to typical dense infiltrates, granulomatous tissue also appears, resembling laryngeal papillomas in its appearance.

The lumen of the larynx is significantly narrowed by annular stenosis, breathing becomes noisy, hissing, and dyspnea occurs during physical exertion. The epithelium covering the infiltrates does not ulcerate (an important differential diagnostic sign), it is covered with a tightly adhering whitish-turbid secretion that emits a sweetish-cloying odor (not fetid, as with ozena, but quite unpleasant).

Diagnosis of laryngeal scleroma

Diagnosis of advanced forms of laryngeal scleroma is not difficult, especially when similar lesions are simultaneously detected in the nasal cavity and pharynx. The scleroma is also given away by the above-mentioned characteristic sweetish-cloying odor, felt at a distance. If scleroma foci affect only the larynx, they should be differentiated from other specific diseases of the larynx and tumors. Along with various methods of comprehensive examination of the patient (x-ray of the lungs, serological tests, bacteriological examination), a biopsy is mandatory in making a final diagnosis. The material must be taken during direct laryngoscopy or even in some cases during dissection of the thyroid cartilage, from the depth of the infiltrate, since due to its density, during indirect laryngoscopy the instrument usually slides along the surface of the mucous membrane and does not penetrate deep into the object.

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Treatment of laryngeal scleroma

Non-surgical treatment of laryngeal scleroma is practically no different from that of rhinoscleroma. The peculiarity of treatment of laryngeal scleroma is its focus on eliminating laryngeal stenosis and ensuring natural functions of the larynx. For this purpose, methods of endolaryngeal surgery, galvanocautery, diathermocoagulation, and methods of dilatation of narrowed parts of the larynx are used. However, the effectiveness of these methods is not high enough due to constant relapses. In case of severe stenosis, a tracheostomy is applied, after which the scar tissue is removed either by endolaryngeal access or by access through the laryngofissure with subsequent plastic surgery using flaps from the local mucous membrane according to B.S. Krylov (1963).

Prognosis for laryngeal scleroma

The prognosis for life with laryngeal scleroma is favorable, but in relation to the functions of the larynx it depends on the severity of the process. Often such patients need multiple plastic surgeries and even become lifelong cannula wearers.