Noma

NOMA, noma (from Greek nome - spreading), water cancer (cancer aquaticus). The name noma, or Wasserkrebs, Watterkrankheit was given by Van der Voorde in the 17th century. The name "water cancer" was given in connection with the watery, glassy infiltration of tissues and the significant destruction of tissue integrity that characterizes Noma as a moist gangrenous process. Some authors believe that Noma, stomatitis ulcerosa, stomacace, and cheilocace are the same process at different stages of development; according to other authors, Noma is a disease identical to hospital gangrene. Noma is most often encountered in childhood (at the age of 2 to 12 years) and is significantly rarer in adults. Infants apparently rarely contract Noma. Cases of Noma in old age have been described. Noma is found mainly in severely emaciated, poorly nourished, and disease-weakened individuals. The cause of Noma should be considered an infection, although there are authors who view Noma as anemic necrosis (Krasin), or as a trophoneurosis (Voronikhin), marantic necrosis (Chernyakhovsky), or traumatic damage to the mucosa with the addition of infection and vascular thrombosis (Oppel). As causative agents of Noma, a special type of Streptothrix, diphtheria bacilli, Vincent's bacillus, a type of Leptothrix, and oral cavity bacteria very similar to Spirillum sputigineum and Spirochaeta dentium are described; some authors have found pyogenic bacteria, staphylococci, especially streptococci in combination with pseudodiphtheritic bacilli and diplococci. Korsh, Flerov, Opokin, and Stepanov found B. fusiformis, s. Vincenti, with spirilla; Zuber classifies Noma in the group of "fusospirillaires" diseases. Schimmelbusch isolated a short bacillus with rounded ends that does not stain by Gram; inoculation of this bacillus caused gangrene only in chickens. Babes and Zambilovici isolated a small, thin bacillus, the injection of an emulsion of which caused tissue necrosis at the injection site in a rabbit. Pomyalovsky isolated a bacillus which, upon injection, caused a creeping ulcer at the injection site in white mice, rabbits, and guinea pigs. Of all the listed causative agents of Noma, fusospirillary types of bacteria are the main etiological factor; their significance in the emergence of Noma must be recognized as almost indisputable, while all other microorganisms are apparently secondary inhabitants of necrotic tissues. Zinserling, who studied the pathology of infectious fusospirochetal gangrene, believes that "in the etiology of infectious gangrene, the main role obviously belongs to the spirochete and B. fusiformis, two bacteria that are well known as common saprophytes of the oral cavity, constantly found in dental plaque (Muhlens), and less often on the tonsils (Schmitz)." In his cases of Noma, Zinserling noted the presence of gangrenous gingivitis and spirochetes. The second starting point of gangrenous infection is the intestine, and from this same source, the area of the anus is apparently infected, and perhaps the genital organs as well, although with genital infection, the transfer of infection directly from the oral cavity is possible, confirmation of which can be found in cases of noma genitalium developing after stomatitis or diphtheria (Zinserling). Pathological-anatomical picture. Upon microscopic examination of Noma, there is no clear demarcation at the border of necrosis and living tissues; a "leukocytic wall is absent, and in general, the inflammatory reaction in the surrounding tissues is weak, and edema prevails in the entire picture. Blood and lymphatic vessels are usually filled with fibrin. The microscopic picture of the dying and dead areas, disintegrating into formless foul-smelling masses, presents the following features: all tissues turn into a structureless mass, cells are in different stages of necrobiosis, their boundaries are unclear, they stain poorly, and nuclei are mostly absent or are in a state of disintegration into chromatin granules. Blood vessels in the dead mass are thrombosed and compressed, their contours are unclear, and muscle fibers are in a state of degeneration and disintegration. In the area of the dead mass, crystals of precipitated hematoidin are encountered. In cases where the further progression of the process is halted, the gradual sloughing of dead tissues occurs, followed by the development of granulations and scarring, and depending on the depth of tissue damage, various forms of facial disfigurement and functional disorders result during scarring. When only the mucosa is affected, scars are formed which, when attempting to open the jaws, are visible from the mouth as white cords, more or less limiting the movement of the jaws depending on the depth of their location. Usually, this form is rarely encountered: most often, the necrotic process, gradually capturing the mucosa, passes to the closely connected buccinator muscle and adipose tissue with facial muscles, leaving the masseter muscle untouched. The skin is the last of all layers to be affected, and the spread of necrosis on it does not reach the magnitude of the necrosis on the mucosa. From the side of the oral cavity, the gums, the bony walls of the alveolar processes, or the entire alveolar process with sockets and teeth perish sequentially. The parotid gland with its excretory duct, like the masseter muscle, is usually not affected by the necrotic process and does not suffer functionally. Factors contributing to the emergence of Noma include measles, typhoid fever, syphilis, diphtheria, dysentery, tuberculosis, poor hygienic living conditions, and stomatitis of various origins, primarily mercurial stomatitis. The first place among infectious diseases after which Noma develops is occupied by measles, especially in combination with tuberculosis and whooping cough (Neuburger). According to Skvortsov (autopsy data), Noma was observed in 5% of cases with measles, in 3% with dysentery, while with scarlet fever, Noma is a great rarity. All these factors significantly weaken the organism and, as it were, prepare the ground for the penetration of the infectious agent that causes Noma. Lexer believes that "the process itself (noma) begins mostly on the basis of inflammatory changes in the mucous membrane of the oral cavity." The infectious origin of Noma is currently almost unquestioned, and this process is now viewed as a putrid infection causing phenomena of gangrenous necrosis. Noma develops most often on the cheeks, less often on the gums, palate, lips (stomatonecrosis, gangraena oris), and in the outer and middle ear. An analogous process (Lexer) is also observed in the area of the anus and on the external female genitalia. Clinically, the process begins with the appearance of a small vesicle with cloudy contents or a superficial ulceration on the mucosa, which then gradually turn into a gangrenous process, spreading first along the surface and then into the depth. This entire process is accompanied by infiltration of the surrounding tissues. When localized in the cheek area, swelling of the latter appears without particular pain, then a dark blue spot appears on the outside of the cheek, which initially does not have inflammatory redness around it. This spot corresponds in its localization to the position of the infiltrate in the area of the mucous membrane. Subsequently, an inflammatory reaction appears around this spot. In the majority of cases, this dark spot leads to perforation of the cheek within the first week. The gangrenous process usually continues further and gradually, depending on the severity of the process, destroys not only the soft parts of the cheek but also the mucous membrane of the upper and lower jaw, loosening the teeth and causing them to fall out, and subsequently exposing the bones of the upper and lower jaw simultaneously. In severe cases, the gangrenous process spreads to the nose, tongue, palate, lips, and the opposite cheek. The process very rarely begins simultaneously on both sides. The course of the process itself is accompanied by high temperature, often clouding of consciousness, with profuse salivation and a large discharge of foul-smelling putrid fluid, the swallowing of which causes severe gastrointestinal disorders, and entry into the respiratory tract often causes the development of aspiration lobular pneumonia and gangrene of the lungs. Opokin distinguishes acute (several days), subacute, and chronic forms of the course of Noma. Mortality from Noma is estimated by various authors at 70% to 95.4% (according to old statistics from 1836, even 100%). According to Lexer, death occurs in almost 75% of cases at the end of the first week, either from heart failure, general exhaustion, or pneumonia. In cases where the process is limited to the necrosis of the affected area, defects of the cheek remain, both through-and-through and non-through. With through-and-through defects, the oral cavity is open, the tongue protrudes, and the teeth bulge outward and sometimes diverge radially, disrupting the correctness of the bite. The edges of the skin and mucosal defect are soldered to the jaws, but usually, the movements of the latter are performed normally. In the front, the lips are pulled down and up by scars toward the wing of the nose; from above, when the process spreads to the orbit, ectropion of the lower eyelid is observed; from behind, the scars usually reach the edge of the masseter muscle, the edge of which is visible through the scarred skin; along its edge on the skin or mucosa, the excretory duct of the parotid gland opens.

Due to the improper position of the duct orifice, as well as deformed lips, constant salivation is observed, which is unusually distressing for patients. In cases of non-penetrating defects, the skin is preserved entirely or almost entirely, with greater or lesser damage to the corners of the mouth and lips; on the side of the oral cavity, in the vestibule, powerful scars are formed, the density of which varies; their ossification is often observed. Depending on their localization, their cicatricial contraction of the jaws can reach such dimensions that any movement, even lateral, is impossible: the act of chewing and digestion is sharply impaired; one has to pour crushed, semi-liquid, or liquid food into the mouth through gaps between the teeth or into the buccal pocket of the healthy side and from there push it into the oral cavity; sometimes, to create a gap between the teeth, it is necessary to remove 1-4 teeth; chewing movements are replaced by movements of the tongue. In connection with the disturbance of the first act of digestion, a disease of the gastrointestinal tract develops, which affects the general condition of the organism (cachexie buccale). Prophylaxis consists of thorough and careful care of the oral cavity in patients, with special attention paid to carious teeth. Therapeutic measures: rinsing with Sol. Kali hypermang., hydrogen peroxide, Acid. borici, a 1% solution of copper sulfate (Golyanitsky), etc. In addition, coloring solutions (gentian violet), Oleum Terebinthinae, sublimate, potassium chlorate, injections of antidiphtheritic serum, injections of neosalvarsan, and red light treatment are used. General treatment: cardiac stimulants, good nutrition, nutrient enemas. All these methods do not always yield satisfactory results. Combined methods of treatment include the following. Kisel's method consists of scraping off dead tissues with a sharp spoon to the limits of healthy areas, smearing this area with iodine, rubbing iodoform powder into it, tamponading with iodoform gauze, and washing the cavity with a hot (up to 40°) solution of potassium permanganate 1.0:4000.0. Scraping is performed 1 or 2 times during the entire treatment period, the remaining procedures 2 times a day. The results of the treatment are quite satisfactory. Golyanitsky's method consists of cauterizing the ulcer with a 10% solution of copper sulfate, rinsing the mouth with a 1% solution of copper sulfate, and applying a compress of a 1% solution of copper sulfate externally. Subsequently, as the process progresses, a prophylactic opening of the salivary duct should be added; in the presence of sequestration of dead tissues, excision of these tissues is added; finally, in the regeneration period, in the presence of a granulation process, it is necessary to continue the use of copper sulfate solutions, without which relapses are not uncommon. Cauterization of dead areas with a hot iron or a Paquelin cautery is used, with the cheek being burned through within the limits of the dead area; it is advisable to make a preliminary transverse incision of the cheek (as proposed by Trendelenburg) in order to expose the entire area. Ranke and Springer recommend early radical surgery and suggest removing the gangrenous area with a wide arcuate incision within healthy tissue and suturing the edges of the wound, which is a far from safe procedure. Any plastic surgery on inflamed tissues is doomed to failure, and one must wait for complete scarring. In cases of cicatricial contraction, systematic expansion of the jaws through the use of wooden, cork, and rubber wedges, screws, and other mouth gags provides relative success in mild cases; with powerful scars, even with their preliminary dissection, no success is observed. To create some movement, an osteotomy of the lower jaw has been proposed in the middle of the ascending ramus, at the angle, in the middle of the horizontal ramus, and along the midline. With this method, frequent relapses are observed; the bones at the site of the cut, despite systematic exercise of the new joint through movement, grow together again, and sometimes, when the peripheral end is pulled backward, an incorrect fusion is created, giving the face an ugly shape. This method is not safe, as purulent osteomyelitis of the lower jaw sometimes develops, which can lead to a fatal outcome (Esmarch). More advisable is the excision of scar masses: without cutting the skin of the cheek (to avoid the formation of new scars), the scars are excised from the side of the oral cavity from the corner of the mouth to the ascending process. It is possible to open the mouth up to 5-6 cm; greater opening is not always achieved due to stiffness in the jaw joint. The defect formed in the oral cavity, to avoid relapse, must be replaced with epithelialized tissue, for which a skin flap is cut on the neck (Bobrov, Almazova), or, in order to avoid the formation of new scars, at a distance on the chest, arm, or back, using the principle of a migrating tubed flap for this purpose. The flap is passed through an opening made under the base of the flap (Bobrov), or through a special opening made on the cheek in front of the masseter muscle, into the oral cavity, where it is applied with its raw side to the cheek at the site of the mucosal defect formed after the excision of the scars; the tip of the flap is folded and used to cover the defect of the cheek and the corner of the mouth. Upon the flap taking root, the pedicle is cut off, and its posterior part is used to replace the mucosa in the depth near the ascending process of the jaw. The oral opening is restored from the remnants of the lips and the folded flap. The skin becomes mucosalized but retains its structure, not metaplasing into mucosa. Instead of a whole skin flap on a pedicle, Esser suggests transplanting skin flaps according to Thiersch; to protect the transplant from infection and allow it to adhere closely to the wound surface formed after the removal of the scars, the author fills the cavity with a hardening mass, which accurately takes the shape of the wound cavity, and then covers the entire surface of this mold with epidermis flaps. After 10 days, the mold is removed, and the epidermis flaps, which have already taken root on the wound surface, remain on the mucosal defect.

In cases of penetrating defects of the cheek, when there is no jaw contracture and the entire cheek and part of the lips are completely absent, plastic surgery is reduced only to restoring the shape of the face. Small defects are closed with mobilized remnants of the lip and cheek elements. Particularly suitable in such cases is a method consisting of mobilizing the lip and healthy cheek from the side opposite to the defect with two parallel incisions under the nose and along the chin fold to the edge of the opposite masseter muscle; the resulting flaps are pulled to the site of the defect in the opposite cheek and sutured to the edges of the defect; subsequently, the operation is supplemented by stomatoplasty. With large defects, it is necessary to restore the mucosa and skin of the cheek. Only in rare cases, in the absence of teeth and the alveolar process, is it possible to cut a flap from the mucosa of the floor of the mouth and tongue according to Lexer. Usually, one has to transplant a folded skin flap, one side of which is used to form the mucosa. The old method of Israel, in which a long skin flap cut on the neck is sutured to the edges of the mucosal defect and, after it has taken root and the pedicle has been cut, is folded in half and placed on the mucosa formed from it, yields frequent failures, since the part of the flap that goes to form the mucosa is poorly nourished, and therefore marginal necrosis with secondary jaw contracture occurs. The most commonly used methods are those in which small skin flaps are taken along the edge of the defect to form the mucosa, the pedicle of which consists of skin or only subcutaneous tissue (Gersuny); powerful skin flaps with large blood vessels nourishing them are thrown onto the raw surface of the latter (a flap from the temporal region according to Lexer and a flap from the cheek with a base at the edge of the lower jaw according to Esser). A penetrating defect can also be easily filled immediately with a Filatov migrating tubed flap constructed at a distance from the defect on the chest, back, or abdomen. V. M. Mysh suggests using a flap from the submandibular region without folding it, as he believes that the raw side quickly epithelializes if the flap is viable. In cases of scarring of the affected Noma region of the genitals, standard plastic measures are applied. M. Ginsburg, L. Nisnevich.