Papillomatosis of the larynx

Laryngeal papillomatosis (papilloma) is a benign tumor that develops from flat or transitional epithelium and protrudes above its surface in the form of a papilla. Papillomatosis is a pathological process characterized by the formation of multiple papillomas on any area of the skin or mucous membrane. Laryngeal papillomas are almost as common as laryngeal polyps. They are the result of a proliferative process that develops in the epithelium and connective tissue elements of the mucous membrane of the larynx.

Solitary papillomas are very rare, in the vast majority of cases these are multiple formations that can occur not only in the larynx, but also simultaneously on the soft palate, tonsils, lips, skin, and mucous membrane of the trachea. Probably, due to the special predisposition of the epithelium, papillomas recur very often, which is why this disease is called papillomatosis.

Papillomas occur most often in early childhood and rarely in adults. Cases of congenital papillomas have been described.

In most cases, papillomas have a viral etiology, which has been proven by a number of authors who managed to reproduce this tumor by autoinoculation of its filtrate. It is also believed that papillomatosis is a kind of diathesis, which manifests itself only in some people with an individual predisposition to it. The role of androgenic hormones in the occurrence of this disease cannot be ruled out, which can probably explain its occurrence only in boys. A number of authors see uneven age-related development of various tissues in the pathogenesis of papillomatosis, which make up the morphological basis of papilloma.

Structurally, papillomas are formations consisting of two layers - papillary connective tissue and epithelial. In multiple papillomas of children, connective tissue, abundantly vascularized elements predominate, while in "older" papillomas in adolescents and adults, elements of the integumentary epithelium predominate, and the connective tissue layer is less vascularized. Such papillomas, unlike the first pink or red ones, have a whitish-gray color.

ICD-10 code

D14.1 Laryngeal papilloma.

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Epidemiology of laryngeal papillomatosis

In the structure of benign tumors, papillomas make up 15.9-57.5%, according to different authors. The disease can begin both in childhood and in adulthood. Juvenile papillomatosis is more common (87%), the symptoms of which appear in the first five years of life.

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Causes of laryngeal papillomatosis

The causative agent of the disease is the DNA-containing human papillomavirus of the papillomavirus family of types 6 and 10. To date, about 100 types of this virus have been identified.

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Pathogenesis of laryngeal papillomatosis

The disease is characterized by a rapid course, a tendency to relapse, often accompanied by stenosis of the larynx. In adults, papilloma develops at 20-30 years of age or in old age. Frequent relapses require repeated surgical interventions, due to which in most cases patients develop cicatricial deformations of the larynx, sometimes leading to a narrowing of its lumen and deterioration of the voice function. In children, bronchopneumonia may develop, and the spread of papillomas to the trachea is diagnosed in 17-26% of cases, to the bronchi and lungs - in 5% of cases. The latter is considered an unfavorable prognostic sign for malignancy.

The disease is accompanied by a decrease in general and local immunity, a violation of its humoral link, and changes in hormonal and metabolic status.

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Symptoms of Laryngeal Papillomatosis

The main clinical sign of laryngeal papillomatosis is hoarseness and breathing problems. The severity of the disease is due to frequent relapses, which can lead to laryngeal stenosis, the possibility of papillomas spreading to the trachea and bronchi with subsequent development of pulmonary insufficiency and malignancy.

Symptoms of laryngeal papillomatosis are determined by the patient's age, localization and prevalence of tumors. Diffuse forms are more common in young children, while papillomas with more limited localization (papillomatosis circumscripta) occur in older children. Papillomas on the vocal folds, characterized by hyperkeratosis, are more common in adults.

The main symptom in both children and adults is increasing hoarseness of the voice, reaching complete aphonia. In children, breathing problems, shortness of breath during physical exertion, and other hypoxic hypoxia symptoms also increase. Dyspnea symptoms increase, laryngeal spasms, stridor, and suffocation syndrome appear, which can result in the child's death if emergency measures are not taken.

In some cases, attacks of asphyxia occur suddenly during a banal intercurrent inflammatory disease of the larynx, developing with concomitant edema. The younger the child, the more dangerous these attacks are, which is due to the significant development of loose connective tissue in the subglottic space, small size of the respiratory tract and the fact that in small children papillomatosis is diffuse and develops very quickly. All these risk factors for asphyxia should be kept in mind when observing such children. In adults, attacks of suffocation are not observed, and the only symptom indicating the presence of a space-occupying formation in the glottis area is hoarseness of the voice.

Classification of laryngeal papillomatosis

There are several histological and clinical classifications of papillomatosis. According to the time of onset of the disease, there are:

• juvenile, arising in childhood;
• recurrent respiratory.

According to the classification of D. G. Chireshkin (1971), the following forms of papillomatosis are distinguished according to the prevalence of the process:

• limited (papillomas are localized on one side or are located in the anterior commissure with the glottis closed by no more than 1/3);
• widespread (papillomas are localized on one or both sides and extend beyond the inner ring of the larynx or are located in the area of the anterior commissure with closure of the glottis by 2/3);
• obliterating.

According to the course of the disease, papillomatosis is divided into:

• rarely recurring (no more than once every 2 years);
• frequently recurring (1-3 times a year or more).

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Screening

All patients with hoarseness and stridor require laryngoscopy and endofibrolaryngoscopy.

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Diagnosis of laryngeal papillomatosis

The laryngoscopic picture can vary greatly.

In rarer cases, isolated small formations from a millet grain to a pea in size are observed, located on one of the vocal folds or in the anterior commissure, reddish in color. In other cases, papillomas have the appearance of cockscombs located on the upper and lower surfaces of the vocal folds; such forms are more common in adults. In young children, in whom laryngeal papillomatosis is most common, diffuse forms of this formation are observed, in which papillomas have the appearance of cone-shaped formations that dot not only the walls of the respiratory slit, but also the adjacent surfaces of the larynx, even extending beyond its limits into the trachea and pharynx. These forms of papillomatosis are well vascularized and are characterized by rapid development and recurrence. With significant sizes, parts of the papillomas can break off during coughing fits and be coughed up with sputum, slightly stained with blood.

The evolution of the disease is characterized by the progression of the proliferative process with penetration into all free cavities of the larynx and, in untreated cases, ends with attacks of acute suffocation, requiring emergency tracheotomy.

Diagnosis in children is not difficult, diagnosis is made using direct laryngoscopy based on the characteristic external signs of the tumor. For differential diagnosis, a mandatory biopsy is performed. In children, laryngeal papillomatosis is differentiated from diphtheria, false croup, foreign body, and congenital malignant tumors. In case of laryngeal papillomas in mature individuals, oncological alertness should be observed, since such papillomas, especially the so-called hard papillomas of a whitish-gray color, have a tendency to malignancy.

When collecting anamnesis, attention should be paid to the frequency of relapses of the disease.

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Laboratory research

General clinical studies are carried out in accordance with the patient's preparation plan for surgical intervention, and the immune status is assessed.

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Instrumental research

All patients should undergo endofibrolaryngotraceobronchoscopy to detect papillomatosis of the trachea and/or bronchi, as well as X-ray and tomographic examination of the lungs.

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Differential diagnostics

Microlaryngoscopy shows a very characteristic picture of papillomatosis - the formation looks like limited, often multiple papillary growths with a fine-grained surface and resembles a mulberry in appearance. Its color depends on the presence of vessels, the thickness of the layer and keratinization of the epithelium, so the papilloma can change color at different periods of its development from red, pale pink to white. Differential diagnostics are carried out with tuberculosis and laryngeal cancer. Signs of malignancy are ulceration of papillomas, changes in the vascular pattern, a sharp limitation of the mobility of the vocal fold in the absence of a cicatricial process, immersive growth, keratosis. Difficulties in differential diagnostics are presented by papillomas in elderly patients and patients with a large number of surgical interventions in the anamnesis. The final diagnosis is established by histological examination.

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Indications for consultation with other specialists

Consultation with an immunologist is recommended.

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Treatment of laryngeal papillomatosis

Treatment goals

• Elimination of airway stenosis.
• Reducing the number of disease relapses.
• Preventing the spread of a process,
• Restoration of voice function.

Indications for hospitalization

Hospitalization is carried out for the purpose of surgical treatment.

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Non-drug treatment of laryngeal papillomatosis

Recently, photodynamic therapy has become widely used.

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Drug treatment of laryngeal papillomatosis

An important role is played by the treatment of postoperative laryngitis - antibiotic therapy, local and general anti-inflammatory therapy. Local use of cytostatics, antiviral drugs and drugs that affect the level of estrogen metabolites, etc. is acceptable. Based on the study of the immune status, immunocorrection is carried out.

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Surgical treatment of laryngeal papillomatosis

The main method of treating laryngeal papillomatosis is surgical. Endolaryngeal removal of papillomas is possible under general or local anesthesia with direct or indirect microlaryngoscopy, using laser or ultrasound. Careful and gentle removal of papillomas is necessary. The number of surgical interventions should be minimized due to the risk of developing laryngeal scarring.

According to N. Costinescu (1964) and a number of other authors, since the etiology of the disease is mainly at the level of hypotheses, numerous proposals for non-surgical treatment of laryngeal papillomatosis turned out to be either ineffective or harmful. By the end of the 20th century, not a single absolutely effective etiotropic treatment had been developed, while existing methods, effective for the most part only in the hands of authors, when used on a mass scale prove to be, at best, only delaying the development of papillomatosis, but not eliminating it. Most of these methods can be classified as auxiliary, used after the use of destructive techniques aimed at the physical elimination of the tumor. However, the "bloody" extirpation of papillomas does not aim to cure this disease, but only to create conditions for more or less satisfactory functioning of the larynx and, in particular, to prevent obstruction of the respiratory tract in children and asphyxia. Repeated surgical interventions are performed in case of relapses, which occur more often and more intensely, the younger the child. In the middle of the 20th century. Papillomas were removed using specially adapted forceps during indirect (in adults) and direct (in children) laryngoscopy. With the development of microsurgical video technology, surgical interventions have become more gentle and effective, but this method does not prevent relapses. With the development of laser surgery, treatment of laryngeal papillomatosis has become significantly more effective, and relapses are rarer and less intense.

As recommended by V. Steiner and J. Werner, before the laser surgery procedure, the beam can be slightly defocused for a softer energy impact on the larynx structures. For this purpose, a low-energy carbon dioxide laser is used. Surgical intervention should be limited to the tumor localization, and the islands of normal mucous membrane located between individual removed papillomas should be preserved as centers of future epithelialization. Papillomas should be removed quite radically, but within the limits of their "fusion" with the underlying tissues to reduce the risk of relapse. Particular care should be taken when operating on bilateral papillomas located in the anterior commissure, since this is where adhesive processes are possible, leading to fusion of the anterior parts of the vocal folds. The authors recommend, especially when operating on children, leaving small areas of papilloma in this area to reduce the risk of an adhesive process. The patient can be extubated immediately after anesthesia, even after removal of large papillomas. To prevent postoperative edema, the authors recommend a single administration of a certain dose of corticosteroid, for example, 3 mg/kg of prednisolone.

Among the recommendations for adjuvant therapy in the postoperative period, broad-spectrum antibiotics, estrogens, and arsenic preparations deserve attention. It was also noted that the administration of methionine after surgery at a dose of 0.5 g 3 times a day for 3-4 weeks prevents relapses. Some authors obtained satisfactory results with subcutaneous administration of placenta extract, while other authors used the tissue therapy method according to Filatov's method, implanting the transplant into the subglottic space via tracheal access. Many authors do not recommend radiation therapy due to possible radiation damage to laryngeal tissues, as well as the risk of malignancy of papillomas.

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Further management

Patients with papillomatosis are subject to mandatory medical examination depending on the frequency of recurrence of the disease, but not less than once every three months.

With surgical treatment, the period of incapacity for work is 7-18 days. With the development of cicatricial deformation of the larynx and trachea, disability is possible

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Information for the patient

If laryngeal papillomatosis is detected, it is necessary to adhere to the terms of dispensary observation, avoid upper respiratory tract infections, vocal strain, and work in dusty, gas-polluted rooms.

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Prevention of laryngeal papillomatosis

Preventive measures are limited to dynamic monitoring of the patient, patient compliance with a gentle voice regimen, elimination of occupational hazards, treatment of concomitant pathology of the gastrointestinal tract (reflux esophagitis) and respiratory tract, inflammatory diseases of the ear, throat and nose.

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Prognosis for laryngeal papillomatosis

The prognosis is usually favorable, even with repeated surgical treatment with the occurrence of postoperative relapses, since as the patient matures, relapses become less frequent and less intense, and then stop altogether. In adults, papilloma may degenerate into cancer or sarcoma, and then the prognosis depends not on the primary disease, but on its complication.

The prognosis of the disease depends on the prevalence and frequency of recurrence of the process. As a rule, it is not possible to completely restore the voice function. The prognosis of the disease is worse after tracheostomy and radiation therapy. Laryngeal papillomatosis is considered a precancerous disease, malignancy occurs in 15-20% of cases, but spontaneous remission is possible.

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