Deforming polyposis of the nose

The deforming polyposis of the nose is a particular form of nasal polyposis, found predominantly in young people, also called Vaquez syndrome.

[1], [2], [3], [4], [5]

The cause of the deforming polyposis of the nose

The cause of development is not fully understood. At different times, it was assumed that the genetic factor, tuberculosis, syphilis is at the heart of this disease, but it was not confirmed. In connection with the development of the doctrine of allergy, the theory of the infectious-allergic origin of the deforming polyposis of the nose is now dominant, supported by the concept of a genetic predisposition to this disease. Observations of V.Racoveanu (1964) showed that if a child or adolescent has polyps of the nose, at which their deforming effect on the rhinorbital region begins to manifest, and if these individuals perform a radical single or multiple removal of polyps, the process of deformation of the nose stops or is not observed . Deformity of the nose is not observed in cases where the polyps of the nose appear after 20 years. Conversely, in children with polyposis of the nose who did not receive the appropriate surgery promptly, the beginning of deformity of the nose progresses despite any non-operative methods of treatment. These and other observations allowed V.Rakoveanu (1964) to make their assumptions about the nature and causes of the deforming polyposis of the nose: in children and adolescents, it is no different from the polyposis of the nose in adults; The only difference is that with deforming polyposis of the nose, polyps appear in childhood; deforming polyposis of the nose arises as a result of mechanical pressure on the compliant, fragile nasal cavity in children and adolescents; the appearance of a deforming polyposis of the nose is also promoted by the neurovegetative and metabolic disturbances observed in these children, which delay the process of ossification of the facial skeleton and thus prolong the effectiveness of the compression effect of expanding polyposic masses on the nasal tissue.

Pathological anatomy and pathogenesis

With deforming polyposis of the nose, intense polypogenesis begins in early childhood and reaches a culmination very early, when the tissues of the nose and face are not yet consolidated. Polypous masses fill all the spaces that lie in their path, partially pushing the tissues of the back of the nose, the frontal processes of the upper jaws, the internal formations of the nasal cavity, filling almost all the paranasal sinuses, and especially the latticed labyrinth, reaching the sphenoid sinus. On their way polyposive masses, putting pressure on young developing tissues, cause their hypotrophy, underdevelopment, resorption. All the changes that arise appear to take on the aspect of polyposis malignant pansinusitis, the external signs of which are manifested by the increase in the nose 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 disruptions in the development of the maxillofacial apparatus (microgenia, upper prognathia, malformation of the occlusion).

Symptoms of deforming polyposis of the nose

Usually patients complain of a complete absence of nasal breathing, pressure in the nose, recurring headaches. The presence of massive dilated polyps in the nasal cavity and its sinuses leads to venous stasis and lymph flow disturbance 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 polyposis of the nose, as a result of compression, atrophy of olfactory receptors arises, because anosmia is not only mechanical, but also neurotrophic irreversible.

The development of deforming polyposis of the nose in childhood due to emerging intracranial discirculatory disorders and hydrocephalus causes children to lag behind in mental and physical development. A characteristic feature of the local process is its progression, manifested in the abundant growth of polyposisations and the constant recurrence of the process, despite the most careful removal of them. The process of polyposis can last for years, decades and even a lifetime, but it is never complicated by malignant polyps, despite their multiple removal. With a prolonged course of the deforming polyposis of the nose, the deforming effect of polyps can reach extreme manifestations even in adulthood. Complications of deforming polyposis of the nose are the same as in the case of banal polyposis of the nose: infectious-allergic mono-, hemi-or pansinusitis, salpingoitis, catarrhal or purulent otitis, etc. An important place among distant complications of the deforming polyposis of the nose is secondary pathological processes in the lower respiratory ways. In the first place among these complications there are respiratory disorders caused by an asthmatic condition, the consequence of which can be chronic bronchitis, pneumonia and their complications. There are also complications from the gastrointestinal tract (aerophagia, bloating, dyspeptic disorders as a result of chronic pyophagy, cholecystitis, pancreatitis, chronic colitis).

Diagnosis of the deforming polyposis of the nose

Diagnosis of the deforming polyposis of the nose in a typical clinical picture does not cause difficulties (history, appearance, anterior and posterior rhinoscopy data, X-ray studies, CT or MRI). Differentiate the deforming polyposis of the nose follows from the banal infectious-allergic polyposis of the nose, polyposis rhinosinusitis. For banal processes, one-sided lesions are most common, the absence of signs of deformation of the skeleton of the external nose, a corresponding anamnesis. The deforming polyposis of the nose should also be differentiated from the juvenile angiofibroma of the skull base, which is characterized by the appearance only in males, fleshy-red, dense consistency, increased spontaneous and when the probe touches the bleeding.

Special difficulties are caused by differential diagnostics in malignant tumors of the latticed labyrinth (sarcoma, cancer) associated with polyposis of the nose. VIVoyachek proved that the formation of polyps in the circumference of the tumor is a consequence of neurotrophic disorders that arise under the influence of the tumor. However, here again, one should pay attention to the increased bleeding of these polyps and the fact that even with multiple histological examination of polyposis, malignant cells are not always detected and the result only indicates the presence of normal polyps of the nose. Distinguishing signs of a malignant tumor of the nose or paranasal sinuses are the early appearance of blood-purulent discharge from the nose with an unpleasant putrefactive odor, pain of a neuralgic nature in the ethmoidaxillar region, and also characteristic data of an x-ray (CT, MRI) study.

[6], [7], [8]

Treatment of deforming polyposis of the nose

Treatment of the deforming polyposis of the nose consists of two parts - the basic (general antiallergic) and symptomatic, which includes medication (appointment of sedatives, analgesic and hypnotics) and surgical, which in principle is also symptomatic (palliative). However, the last, timely begun, in most cases prevents the development of the deforming process, which stops spontaneously at the age of the patient for more than 20 years due to the completion of the development and consolidation of the bones of the facial skeleton.

Prophylaxis of deforming polyposis of the nose

Prevention consists in early detection of polyps of the nose in children, their timely removal, basic antiallergic treatment, sanitation of foci of infection and, in the first place, chronic etmoiditis.