Deforming nasal polyposis

Deforming nasal polyposis is a particular form of nasal polyposis, which occurs mainly in young people, also called Vaquez syndrome.

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Cause of deforming nasal polyposis

The cause of development is not fully understood. At different times it was assumed that the basis of this disease is a genetic factor, tuberculosis, syphilis, however, no confirmation of this was found. In connection with the development of the doctrine of allergy, the theory of infectious-allergic origin of deforming nasal polyposis currently dominates, supported by the concept of genetic predisposition to this disease. Observations of V. Racoveanu (1964) showed that if nasal polyps are found in a child or teenager, in which their deforming effect on the rhinoorbital region begins to manifest, and if these individuals undergo radical single or multiple removal of polyps, then the process of deformation of the nose stops or is not observed. Deformation of the nose is not observed in cases where nasal polyps appear after 20 years. On the contrary, in children suffering from nasal polyposis who did not undergo appropriate surgical intervention in a timely manner, the onset of nasal deformation progresses, despite any non-surgical treatment methods. These and other observations allowed V. Racoveanu (1964) to express his assumptions about the nature and causes of deforming nasal polyposis: in children and adolescents it is no different from nasal polyposis in adults; the only difference is that with deforming nasal polyposis, polyps appear in childhood; deforming nasal polyposis occurs as a result of mechanical pressure on pliable, fragile formations of the nasal cavity in children and adolescents; the occurrence of deforming nasal polyposis is also facilitated by neurovegetative and metabolic disorders observed in these children, which delay the process of ossification of the facial skeleton and thus prolong the effectiveness of the compressive effect of the growing polypous masses on the tissues of the nose.

Pathological anatomy and pathogenesis

In deforming nasal polyposis, intensive polyp formation begins in early childhood and reaches its culmination very early, when the tissues of the nose and face are not yet consolidated. Polypous masses fill all the spaces lying on their way, partially pushing apart the tissues of the bridge of the nose, the frontal processes of the upper jaws, the internal formations of the nasal cavity, filling almost all the paranasal sinuses, and primarily the ethmoid labyrinth, reaching the sphenoid sinus. On their way, polypous masses, exerting pressure on young developing tissues, cause their hypotrophy, underdevelopment, and resorption. All the resulting changes acquire the aspect of polypous malignant pansinusitis, the external signs of which are manifested by an increase in the nasal pyramid at the level of the bone floor. The absence of nasal breathing forces children to constantly be with their mouth open, which leads to disturbances in the development of the maxillofacial apparatus (microgenia, upper prognathia, malocclusion).

Symptoms of deforming nasal polyposis

Patients usually complain of a complete lack of nasal breathing, pressure in the nasal area, and periodic headaches. The presence of massive distending polyps in the nasal cavity and its sinuses leads to venous congestion and disruption of lymph flow not only in the rhinosinus system, but also in intracranial formations, primarily in the venous system of the brain, which, in turn, can lead to external and internal hydrocephalus with all the ensuing consequences. With deforming nasal polyposis, as a result of compression, atrophy of the olfactory receptors occurs, therefore anosmia is not only mechanical, but also neuroatrophic and irreversible.

The development of deforming nasal polyposis in childhood due to intracranial circulatory disorders and hydrocephalus leads to mental and physical developmental delays in children. A characteristic feature of the local process is its progression, expressed in abundant growth of polypous formations and constant recurrence of the process, despite their most thorough removal. The process of polyp formation can last for years, decades and even a lifetime, but it is never complicated by malignancy of polyps, despite their repeated removal. With a long course of deforming nasal polyposis, the deforming effect of polyps can reach extreme manifestations in adulthood. Complications of deforming nasal polyposis are the same as those of common nasal polyposis: infectious-allergic mono-, hemi- or pansinusitis, salpingootitis, catarrhal or purulent otitis, etc. An important place among the distant complications of deforming nasal polyposis is occupied by secondary pathological processes in the lower respiratory tract. In the first place among these complications are respiratory disorders caused by an asthmatic condition, the consequence of which can be chronic bronchitis, pneumonia and their complications. Complications from the gastrointestinal tract are also observed (aerophagia, intestinal distension, dyspeptic disorders as a result of chronic pyophagia, cholecystitis, pancreatitis, chronic colitis).

Diagnosis of deforming nasal polyposis

Diagnosis of deforming nasal polyposis with a typical clinical picture does not cause difficulties (anamnesis, appearance, data of anterior and posterior rhinoscopy, X-ray examinations, CT or MRI). Deforming nasal polyposis should be differentiated from banal infectious-allergic nasal polyposis, polypous rhinosinusitis. Banal processes are most often characterized by unilateral lesions, the absence of signs of deformation of the skeleton of the external nose, and a corresponding anamnesis. Deforming nasal polyposis should also be differentiated from juvenile angiofibroma of the skull base, which is characterized by occurrence only in males, fleshy-red color, dense consistency, increased spontaneous bleeding and bleeding when touched with a probe.

Particularly difficult is differential diagnostics in malignant tumors of the ethmoid labyrinth (sarcoma, cancer) associated with nasal polyposis. V.I. Voyachek proved that the formation of polyps around the tumor is a consequence of neurotrophic disorders that occur under the influence of the tumor. However, here too, attention should be paid to the increased bleeding of these polyps and to the fact that even with repeated histological examination of polypous tissue, malignant cells are not always detected and the result indicates only the presence of ordinary nasal polyps. Distinctive signs of a malignant tumor of the nose or paranasal sinuses are the early appearance of bloody-purulent discharge from the nose with an unpleasant putrid odor, neuralgic pain in the ethmoidomaxillary region, as well as characteristic data from X-ray (CT, MRI) examination.

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Treatment of deforming nasal polyposis

Treatment of deforming nasal polyposis consists of two parts - basic (general antiallergic) and symptomatic, which includes medication (prescription of sedatives, analgesics and sleeping pills) and surgery, which is, in principle, also symptomatic (palliative) in nature. However, the latter, if started in a timely manner, in the vast majority of cases prevents the development of the deforming process, which stops spontaneously at the age of the patient over 20 years due to the completion of the development and consolidation of the bones of the facial skeleton.

Prevention of deforming nasal polyposis

Prevention consists of early detection of nasal polyps in children, their timely removal, basic anti-allergic treatment, sanitation of foci of infection and, first of all, chronic ethmoiditis.