Ozaena

OZAENA, a disease characterized mainly by 1) atrophy of the nasal mucosa (and its accessory cavities), accompanied by marked atrophy of the nasal turbinates, 2) secretions that thicken into crusts, and 3) a foul odor from the nose. This disease was already known among the Egyptians under the name pa'a, and among Indians as putinasya. In recent centuries of the Christian era, a number of names for this disease appear: coryza virulenta or maligna, dysodia, ulcus narium factens, Stink-nase, etc. Until 1873-76, the prevailing view was of O. as an ulcerative process of the nasal mucosa, and only in 1876 Frankel gave the modern definition of O. Further study of O. revealed that a number of diseases similar to it in symptoms were falsely considered as O. Many cases of syphilis, tuberculosis, leprosy, rhinoscleroma, foreign bodies in the nose, and diseases of the accessory nasal cavities were diagnosed as O. At present, there can be no question of the existence of O. syphilitica, tuberculosa, traumatica, etc., as it has been fully established that O. is an independent disease based on deep atrophic processes, the origin of which is still unknown, but probably the same as those of rhinitis atrophica, one form of which O. represents. The etiology of O. remains unclear to this day. There is a whole series of theories: influence of heredity, constitution, and external environmental factors, but none of them fully explain the etiology of this disease. Thus, hereditary influences in atrophic rhinitis are under study, and despite the fact that data supporting the inheritance of this disease are provided by very large clinicians (in particular in our country by V. I. Voyachev), genetic analysis of the presented materials does not yet allow a definite statement in this regard. Data on the influence of constitutional factors on the development of O. are also still controversial. As for the influence of professional and domestic factors, there are only general statistical data, from which it follows that people in poor domestic conditions suffer from O. more often, and that in some professional groups, for example among shoemakers, furriers, and tobacco workers, ozaena is more widespread than among the rest of the population. Geographical distribution of O. Ozaena is by no means found in all countries of the globe. Thus, according to Roy's research, it does not occur at all among blacks in Africa, among Arab tribes on the coast of Africa, while in Central American countries it is quite widespread. It has significant distribution in China, Japan, and India. In Europe, O. is found almost everywhere and most often in Spain (1/3 of all nasal diseases), in Switzerland, in Greece and other Balkan states. In Germany, Italy, Denmark, Austria, the number of O. patients ranges from 1% to 1½% of all nasal diseases. In the USSR, O. is distributed approximately the same as in the countries of Central Europe. According to data from the preventive children's outpatient clinic in Moscow for 1919-23, O. constitutes 0.4% of the registered child population; according to data from the Obukh Institute for 1927-29 - 0.35%. O. affects women predominantly (almost three times more often than men). According to Danish school statistics, O. occurs in boys in 0.31%, and in girls in 0.55% of all examined children (17,653 people). An idea of the distribution of O. by age groups can be obtained from the figures of Demetriades and Moutoussis (Greece), as well as A. I. Feldman (Moscow). Age Demetriades and Moutoussis Feldman abs. number percent abs. number percent 1-10 years 10-20 » 20-30 » 30-40 » 40-50 » 50-60 » 60-70 » 70-80 » 6,02 38,29 28,63 14,43 6,13 3,4 1,36 0,116 12,22 52,77 20,55 9,44 2,77 1,66 The largest number of patients falls in the age group from 10 to 20 years, and then in the age group 20-30 years. Most statisticians give similar figures (approximately). The distribution of O. by social groups can be seen from the statistics of the professional consultation of the Obukh Institute for the Study of Occupational Diseases (Moscow). For 1927-29: among children of workers - 0.35%, employees - 0.28%, pupils of children's homes - 0.26%. Pathological anatomy of O. There is no unified view in the interpretation of the patho-anatomical picture of O. While some find that atrophy of the mucosa results from proliferative inflammation of it (Lautenschlager), others do not find signs of inflammation in O. and consider atrophy a primary process, the essence of which is still unclear. In developed cases of O., macroscopically, atrophy of the mucosa is observed, against which foci of hypertrophied tissue are sometimes noticeable. Lautenschlager interprets these changes as different stages of the inflammatory process. Histologically, he found the integrity of the epithelial covering, but the cylindrical epithelium is replaced by flat on large areas; in the submucous layer, corresponding to hypertrophic foci, round-cell infiltration is observed, especially around vessels and mucous glands. Such an infiltrate is also found in the periosteum, Haversian canals, and bone marrow cavities of the nasal turbinates. In addition, in the submucous layer, granulation tissue proliferation is observed. Subsequently, the round-cell infiltrate disappears, and the granulation tissue matures and undergoes fibrosis. In the mucous glands - fatty degeneration, their ducts are cystically dilated due to the retention of secretion in them. These changes correspond to the atrophic state of the mucosa. The bone of the nasal turbinates is greatly thinned, a large accumulation of osteoclasts is observed around it, and osteoblasts are completely absent. Data on the condition of the vessels are contradictory. According to some (Frankel, Schonemann, Kravise), productive endarteritis is observed in the vessels of the submucous layer, others found proliferation of connective tissue in the adventitia and media of the vessels, and finally, others found no changes in the vessels at all. Opposite to Lautenschlager, Frankel, Schonemann, and Oppikofer did not always find metaplasia of the epithelium. The descriptions of changes in the accessory nasal cavities made by many with the aim of clarifying their connection with the development of ozaena are very interesting. According to Lautenschlager, the epithelial covering of the sinus mucosa is well preserved and little metaplastic. The basement membrane is often hyalinized. Round-cell infiltration is not always present. In the deep layers of the mucosa around the submucous glands, there is proliferation of connective tissue, which undergoes hyalinization. The mucous glands are either degenerated and their ducts are cystically dilated, or opposite phenomena of hyperplasia of the glands and their ducts are observed, which are strongly tortuous and twisted. The vessels are sclerosed due to the proliferation and hyalinization of connective tissue, mainly in the adventitia, but often in all three tunics of the vessel. The bone, especially the facial wall of the maxillary cavity, is sclerosed and often thickened. Thus, Lautenschlager's research shows that in ozaena, there is very often a hyperplastic inflammatory process in the accessory nasal cavities, even when there is already a well-expressed atrophy of the mucosa and bone in the nasal cavity. The character of nasal cavity secretions has been studied in considerable detail. In the initial stages, the secretion is liquid, more or less sticky, stringy, and when it dries, forms small crusts. The secretion mainly accumulates in the middle nasal passage, from where it flows down the surface of the inferior turbinate to the bottom of the nose. To the pure secretion of yellowish-white color, resembling pus, particles of dust, elements of blood are mixed in, and then it takes various shades: gray, green, yellow. The longer O. lasts, the thicker the secretions become; they dry into dense crusts, upon removal of which the mucosa sometimes bleeds. On the surface of the mucosa, there is usually a more liquid layer of secretion that does not emit an odor. Upon microscopic examination, a large number of pus cells, flat epithelial cells, fat-free cells, and many microorganisms are found. The reaction of the secretions is strongly alkaline. The secretions contain, in addition to the usual substances of mucus, a number of odorous compounds: indole, skatole, phenol, hydrogen sulfide, and fatty acids. Pathogenesis of O. There is no generally accepted view on the pathogenesis of O. There are many theories explaining the origin and essence of this disease. One of the most extensive groups of theories is based on the concept of O. as a primary disease of the nasal mucosa, which subsequently leads to the atrophy of the bony parts of the nose. Some (Frankel, Voltolini, and Schonemann) believe that primarily a catarrhal inflammation plays out in the nasal mucosa, spreading to the deep layers and passing to the submucous layer. The bone becomes inflamed secondarily. Under the influence of still unknown toxins, the inflammation leads to the specific atrophy of the nasal mucosa and odor characteristic of O. Others (Abel, Krause, Schaffer, Lermoyez) attribute the main role in the occurring inflammation to the Bacillus mucosus found by Abel - Lowenberg, Perez - Coccobacillus foetidus ozaenae, and Bacillus foetidus liquefaciens.

A large group of researchers consider the primary change in the mucosa in O. to be the metaplasia of cylindrical epithelium into flat epithelium. According to the opinion of many, an abnormally large width of the nasal cavity predisposes to the development and progression of atrophy. According to this view, in a wide nose the flat epithelium cornifies more quickly and atrophy of the underlying layers of the mucosa occurs more rapidly. The vessels become empty, and as a result the bony skeleton of the turbinates also undergoes atrophy. Zaufal expresses the hypothesis that the atrophy of the mucosa in the excessive width of the nasal cavity is also contributed to by the fact that the secret dries quickly due to the large masses of air, but due to the weakness of the air current movement in the dilated nose, it poorly separates from the underlying mucosa. Walb attributes special importance to this circumstance, believing that crusts cause atrophy of the mucosa from pressure. The theory of epithelial metaplasia raises the most serious objections, since often cases of O. are encountered where epithelial metaplasia is expressed very weakly or is even completely absent. At the same time, there are quite a few diseases of the nasal mucosa where epithelial metaplasia is expressed very sharply, but the sense of smell is absent. The width of the nose apparently also does not serve as a cause for the development of O., since many authors (including A. I. Feldman in Moscow) note that O. develops almost equally often in wide and narrow noses. Finally, there are known facts when entire peoples, differing in the wide structure of the nasal skeleton (e.g., Mongols), suffer from O. very little. At the basis of another group of theories of the pathogenesis of O. lies the concept that primarily affected is not the mucous membrane, but the bones of the nasal turbinates. Cholewa and Cordes assume a disorder of blood circulation in the bony vessels of the nasal turbinates, due to which a disturbance of nutrition of the bone and the associated mucosa develops. Processes of bone dystrophy are also assumed, leading to the loss of calcium salts by the bone, similar to the process of osteomalacia. Zaufal attributes special importance to the congenital small size of the lower turbinates, due to which the reduced surface of the mucosa copes worse with harmful environmental factors. Zarniko explains the development of O. by trophoneurotic disorders in the area of the nervous system of the nose. Trophoneurosis leads to a suppression of the viability of the nasal tissues, as a result of which they atrophy. In his opinion, the bone atrophies first. The experiments of Iri, who induced O. in rabbits, as well as Caldera, who after cutting the 2nd branch of the trigeminal nerve in rabbits inoculated the secret of O. patients, and finally the research of Galotti, who studied the structure of the 2nd branch of the trigeminal nerve in O. patients, have not brought any confirmation in favor of the trophoneurotic theory. The so-called 'focal' theory of Michel-Grunwald, later modified by Lautenschläger, is widely recognized by many modern clinicians. According to this theory, O. develops as a consequence of diseases of the paranasal sinuses. According to Lautenschläger, in the paranasal sinuses under the influence of childhood infections, proliferative inflammation develops. This inflammation directly leads to exudative-productive inflammation of the nasal mucosa. The further development of inflammation of the nasal mucosa leads to atrophic changes not only of the mucosa, but also of the bone. Lautenschläger's views are substantiated by a large number of pathohistological studies and numerous observations of the condition of the nasal cavity and sinuses in O. Despite the apparent simplicity and clarity of this theory, it has such controversial points that do not allow it to be considered entirely correct. No less widespread than the 'focal' theory is the 'infectious' theory, created by Lowenberg, Striibing, Abel, Perez, Hofer and others. Lowenberg was the first to seek a specific causative agent in O. and isolated a special rod from the secret of ozena patients. Abel and Striibing later isolated and bacteriologically studied this same rod, which was named Bacillus mucosus Abel-Lowenberg. However, this rod turned out to be identical with Pneumobacillus Friedländeri, did not produce odor and subsequently lost its significance. Later, the causative agent of O. was seen in the diphtheria rod (Vogel), the pseudodiphtheria rod (della Vedova, Belfanti) and in a whole series of other microorganisms. The bacillus isolated by Perez (Coccobacillus foetidus ozaenae), which turned out to be close in its properties to the rod of Hayek, received significantly greater recognition. This bacillus grows on all media, is Gram-negative, immobile, of a coccobacillus type, polymorphic, mutates broth without forming a film, and forms indole. In liquid cultures, the bacillus develops the characteristic odor of O. Hofer, who studied this bacillus, succeeded in causing an ozena-like disease in dogs by intravenous injection of a culture obtained from ozena patients. From the nasal secret of these dogs, Perez's bacillus was then isolated. In therapeutic testing of serum obtained from dilution of Perez's bacillus, good results were also obtained. Agglutination of the serum in 100 patients gave a positive result. Furthermore, Perez, studying the conditions of transmission of O. infection, came to the conclusion that O. infection is transmitted by contagion from person to person. A dog can be a transmitter. The observations of Perez-Hofer were confirmed by a number of researchers. However, an even larger number of researchers denies the specificity of the Perez-Hofer bacillus for O., since most failed to isolate the culture of bacillus Ozaena Perez from inoculated animals, serodiagnosis of O. was unsuccessful, and finally vaccination did not lead to positive results. The views on the pathogenesis and etiology of O. presented here do not exhaust all existing assumptions on this matter, however, they show how little absolutely reliable there is in all these theories. Depending on one or another view of the origin and essence of the pathological process in O., the origin of the most important symptom of O.-odor-is explained differently. It is known that odor is given off only by drying crusts. Some assume that the odor is caused by the decomposition of the secret under the influence of air microbes; others, that in the secret protein decomposition by putrefactive bacteria occurs; third, that only the secret of Bowman's glands decomposes; fourth, that the cornified epithelium decomposes and its decomposition gives off odor. Finally, many believe that the odor is caused by products of bone decomposition. The course of O. is extremely slow; at the beginning of the disease apparent improvements are often observed, especially during periods of acute rhinitis. Gradually O. spreads to the nasopharyngeal cavity, pharynx and larynx. Symptoms of O. Subjective symptoms are often insignificant, especially at the beginning of the disease. In the first period of the disease there are complaints on nasal obstruction and abundant, thickening discharge from the nose. In the later period of the disease, nasal obstruction with crusts is distressing. When removing crusts by vigorous blowing, there are often small nosebleeds. The sharp weakening, and sometimes complete absence, of the sense of smell greatly distresses patients. A characteristic is the depressed condition of O. patients, who feel that due to their odor they are a burden to those around them. On external examination, sometimes a depression of the nasal bridge, widening of the nostrils, which are often turned more forward than downward, is noticeable. On rhinoscopy in the initial period, a thinned, hyperemic mucosa covered with a sticky, tenacious secret, accumulating in the middle and lower nasal passages, is visible. In far-advanced O., the entire mucosa is covered with grayish-green crusts giving off odor. Sometimes the crusts reach enormous sizes, and when removed, bleeding from the nasal mucosa occurs. The inferior turbinate is always sharply reduced in volume; the middle turbinate is not always atrophied, sometimes on the contrary, it protrudes as a large mass; the entire nose represents a wide tube, divided by the septum into two parts; the posterior wall of the pharynx and details of the lateral wall of the nose are well visible in the nose cleared of crusts, even the cushion of the pharyngeal opening of the Eustachian tube. Diagnosis of O. is made easily on the basis of the characteristic complaints of the patient, rhinoscopic picture, presence of foul-smelling crusts, absence of smell. The difference from syphilis and tbc is the integrity of the epithelium of the mucosa. The prognosis in O. regarding the restoration of the normal condition of the nose is unfavorable, since treatment allows only the most distressing symptoms to be eliminated: odor and accumulation of crusts. In old age (approximately at 50 years) the formation of crusts and consequently odor very often ceases. The sense of smell never recovers. Treatment of O. Despite the exceptionally large number of means proposed for the treatment of O., there is not one that acts radically. Symptomatically effective are all those means that are capable of causing irritation of the nasal mucosa and enhancing its secretion. As mechanically irritating means, massage of the mucosa with a cotton probe, Götzschian nasal packing, inhalations, etc., are used.

Among medicinal substances, the most widespread application is the use of lubrications with Lugeol's solution, and washing the nose with solutions of NaCl, soda, and borax. Inhalation and snuffing of 'sneezing' powders (Rhiz, Iridis, Veratrin), which cause a sharp attack of sneezing, are also used. The effects of sulfur and vaccine therapy have been extensively tested. The results obtained from the use of these treatment methods are no better than those from the medicinal treatment method. Extensive tests were conducted on the polyvalent Hoeffke vaccine, but it also gave almost no positive results. Thus, there is no single therapeutic agent that acts radically in Ozaena. All means that cause irritation of the nasal mucosa in one way or another have a positive symptomatic effect. X-ray and radiotherapy were used without success. The surgical treatment of Ozaena is very diverse. The basis for the idea of surgical treatment of Ozaena lies in the various theories of its origin already described above. Thus, authors, each according to their own point of view, propose different methods of intervention. Removal of the middle turbinate, complete scraping of the nasal mucosa, and removal of the lower turbinate have been performed. Further, after the complete failure of these operations, most authors went in search of ways to narrow the nasal cavity. Saenger and Sondermann inserted special obturators into the nasal openings, many introduced paraffin, petrolatum with barium, and spermaceti under the nasal mucosa. Sturmann introduced cartilage taken from a septum resection, Schonstadt inserted bone from the tibia, Eckert-Moebius introduced macerated bone (tibia) of a calf, Burak warmly recommended fatty tissue. Glass transplants a healthy lower turbinate taken from another person. After all these operations, there is initially a significant revival of the mucosa, depending on the inflammatory process. Following the acute inflammation, everything returns to the original state of atrophy. Larger surgical interventions have been proposed by Lautenschläger for the simultaneous treatment of the paranasal sinuses and the nasal cavity itself. Basically, the operation consists in opening the maxillary and ethmoid sinuses, which are thoroughly scraped. The lateral wall of the nose is pressed into the nasal cavity on almost its entire length from the side of the paranasal sinuses. Sometimes, along with this, bone plastic material, obtained from the facial wall of the maxillary sinus, is introduced between the mucosa of the nasal floor and the bone, as well as under the mucosa of the lateral wall of the nose. Witmaack, based on considerations of the insufficiency of liquid secretion in Ozaena, transplants the excretory duct of the parotid gland into the maxillary sinus. Lautenschläger supplemented this operation with new plastic techniques for wound closure and the transplantation of bone tissue under the nasal mucosa. Halle makes an incision along the entire lateral wall of the nose and brings it to the midline, thereby achieving narrowing of the nose. Hinsberg achieves the same goal by hollowing out the lateral walls of the nose and pulling them to the midline with the help of dense sutures passing through the entire thickness of the lateral walls of the nose and the septum. The result of all these surgical interventions is only temporary improvement. Radical cure is not achieved in this way, and therefore operations for Ozaena are gradually losing a significant number of their supporters. Prevention of Ozaena at the present time should consist mainly in the proper sanitation of the upper respiratory tract among the child population; it is especially necessary to fight against constant childhood colds. In addition to medicinal measures, it is necessary to establish a proper regimen and warmly recommend physical culture measures, especially winter ones. At present, special medical societies in the USSR and abroad maintain strict records of patients with Ozaena and conduct detailed research on these patients. The health authorities of the USSR also conduct dispensary observation and carry out appropriate sanitation. In each individual case of Ozaena, it is absolutely necessary to recommend to the patient the rules of personal hygiene: daily nasal washing, a separate towel, etc.