Cholesteatoma

CHOLESTEATOMA, a pearl tumor consisting of keratin scales and observed in the skin, bone, mucous membranes, and meninges; in the latter case, due to the accumulation of layered masses of keratinized epidermal scales, the tumor often acquires a special luster and whiteness, which is the reason for its designation as X., a pearl tumor. The structure of X. Macroscopically, the structure of X. is presented in Fig. 1. Microscopically, the main mass of X. consists of concentrically arranged polygonal plates of anucleate epidermoid cells, between which cholesterol plates are present to a greater or lesser extent. This mass is surrounded by a Cholesteatom-matrix. The latter consists of an outer and an inner layer. The outer layer, adjacent to the bone, consists of connective tissue, poor in cellular elements, abundant in branching vessels, and containing a small amount of elastic fibers. The inner layer resembles the structure of the skin epidermis. The history of the development of the doctrine on X. For the first time, a tumor-like disease of this order was found by Cruveilhier in the arachnoid membrane of the brain and named tumeur perlee (pearl tumor). Similar tumors were described in 1838 by Müller (J. Müller) and were first named cholesteatomas by him due to their layered structure and fatty luster. The first case of X. of the ear, described in 1838 by Pappenheim as a case of blockage of the tympanic cavity, concerned an 11-year-old child in whom a fatty-scaly tumor the size of a pea was found in the mastoid process, consisting primarily of cholesterol crystals and calcium carbonate. The basis of the formation of X. lies in the proliferation of remnants of embryonic epidermis (Virchow). Based on this theory, it becomes understandable why X. is most often observed in the temporal bone, since the middle ear is formed in it as a result of the regression of gill arches. Thus, the view was established that X. is an epidermoid congenital tumor. However, the fact that in chronic purulent diseases of the middle ear X. is observed very often (40%) (X. without ear disease is a rare phenomenon) forced physicians at one time to consider ear X. as a "false" X., which is a product not of a tumor-like but of an inflammatory order. As a result, the view (Gabermann-Betzold) was established that the Cholesteatom-matrix is epidermis that has invaded from the external auditory canal into the cavity of the middle ear. In the vast majority of cases, the invasion of epidermis from the external auditory canal into the affected mucous membrane of the middle ear occurs through a parietal perforation of the tympanic membrane (see Otitis). X. of the middle ear was also successfully induced experimentally (Berberich): cold or hot resin was injected into the external auditory canal, a perforation was created in the tympanic membrane through paracentesis or cauterization with hot resin; X. formed due to the invasion of excessively regenerating epithelium towards the attic. However, the patho-anatomical substrate is the same in both congenital and otogenic X. It would seem, therefore, that both these forms of X. should have the same genesis. And indeed, the investigations of Manasse showed that the basis of otogenic X. has the same pathogenesis as the basis of the true (congenital) one. According to Manasse, epithelium from the external auditory canal, growing in the direction of the middle ear, does not simply cover the exposed mucosa but has a tendency to intensely grow in the form of tongues, processes, and into the submucosal layer, and, growing, forms a cholesteatoma tumor protruding into the cavity of the middle ear, sometimes covered on the outside by normal mucous membrane. The independent tendency to rapid growth of flat epithelium along the connective tissue bed is the main point for explaining the origin of otogenic X. The difference between true and "false" (ear X.) consists only in the fact that in the first case the displacement of epithelium occurred congenitally, and in the second - during life, caused by an inflammatory process. Considering, however, that not all chronic diseases of the middle ear cause X., one has to consider the question of the development of otogenic X. as open. It is possible that the development of the epidermal surface of X. in the middle ear sometimes proceeds without the invasion of epithelium from the outside ear, but by metaplasia of the epithelium of the middle ear. In any case, this is the mechanism of development of X. in the uterus, gallbladder, in the urinary tract, etc. Differential diagnosis between congenital and otogenic forms of X.: absence in the past and present of inflammatory disease of the middle ear, objective data on its having been suffered, as well as the location of X. in a place far removed from the cavities of the middle ear, approximately in the scale of the temporal bone (Fig. 2), speaks for cholesteatoma verum. X. of the same order is classified as X. in the cerebellopontine angle (Fig. 3). Diagnosis - see Otitis. Suspicious for X. is the finding in the lavage fluid in chronic purulent otitis of epidermal flaps and crumbs that settle to the bottom of the vessel (vessel with a bottom stained black). Ooscopically, X. can sometimes be seen with a sufficient size of perforation in the form of epidermoid masses of a grayish-white color protruding from the perforation hole or the cavity itself. In doubtful cases, a microscopic examination is necessary. The protracted course of cases of purulent disease of the middle ear that proceed acutely can be due to X., which can be detected by serial histological examination of the operative material. Chronic purulent otitis accompanied by headaches with a feeling of heaviness, fullness in the head, dizziness, and sometimes disturbance of the function of the facial nerve, in the vast majority of cases is complicated by a cholesteatoma process. A great diagnostic aid is a special roentgen examination, which sometimes reveals the presence of X. even with insignificant clinical symptoms. Prognosis. Untreated X. leads to death through meningeal or sinusogenous infection. Treatment is exclusively surgical (see Mastoiditis), leading in the vast majority of cases to a cure in the absence of involvement of the meninges and brain substance.

Figure 1.

often (40%) (X. without ear disease is a rare phenomenon), forced physicians at one time to consider ear X. as a "false" X., which is a product not of a tumor-like but of an inflammatory order. As a result, the view (Gabermann-Betzold) was established that the Cholesteatom-matrix is epidermis that has invaded from the external auditory canal into the cavity of the middle ear. In the vast majority of cases, the invasion of epidermis from the external auditory canal into the affected mucous membrane of the middle ear occurs through a parietal perforation of the tympanic membrane (see Otitis). X. of the middle ear was also successfully induced experimentally (Berberich): cold or hot resin was injected into the external auditory canal, a perforation was created in the tympanic membrane through paracentesis or cauterization with hot resin; X. formed due to the invasion of excessively regenerating epithelium towards the attic. However, the patho-anatomical substrate is the same in both congenital and otogenic X. It would seem, therefore, that both these forms of X. should have the same genesis. And indeed, the investigations of Manasse showed that the basis of otogenic X. has the same pathogenesis as the basis of the true (congenital) one. According to Manasse, epithelium from the external auditory canal, growing in the direction of the middle ear, does not simply cover the exposed mucosa but has a tendency to intensely grow in the form of tongues, processes, and into the submucosal layer, and, growing, forms a cholesteatoma tumor protruding into the cavity of the middle ear, sometimes covered on the outside by normal mucous membrane. The independent tendency to rapid growth of flat epithelium along the connective tissue bed is the main point for explaining the origin of otogenic X. The difference between true and "false" (ear X.) consists only in the fact that in the first case the displacement of epithelium occurred congenitally, and in the second - during life, caused by an inflammatory process. Considering, however, that not all chronic diseases of the middle ear cause X., one has to consider the question of the development of otogenic X. as open. It is possible that the development of the epidermal surface of X. in the middle ear sometimes proceeds without the invasion of epithelium from the outside ear, but by metaplasia of the epithelium of the middle ear. In any case, this is the mechanism of development of X. in the uterus, gallbladder, in the urinary tract, etc. Differential diagnosis between congenital and otogenic forms of X.: absence in the past and present of inflammatory disease of the middle ear, objective data on its having been suffered, as well as the location of X. in a place far removed from the cavities of the middle ear, approximately in the scale of the temporal bone (Fig. 2), speaks for cholesteatoma verum. X. of the same order is classified as X. in the cerebellopontine angle (Fig. 3). Diagnosis - see Otitis. Suspicious for X. is the finding in the lavage fluid in chronic purulent otitis of epidermal flaps and crumbs that settle to the bottom of the vessel (vessel with a bottom stained black). Ooscopically, X. can sometimes be seen with a sufficient size of perforation in the form of epidermoid masses of a grayish-white color protruding from the perforation hole or the cavity itself. In doubtful cases, a microscopic examination is necessary. The protracted course of cases of purulent disease of the middle ear that proceed acutely can be due to X., which can be detected by serial histological examination of the operative material. Chronic purulent otitis accompanied by headaches with a feeling of heaviness, fullness in the head, dizziness, and sometimes disturbance of the function of the facial nerve, in the vast majority of cases is complicated by a cholesteatoma process. A great diagnostic aid is a special roentgen examination, which sometimes reveals the presence of X. even with insignificant clinical symptoms. Prognosis. Untreated X. leads to death through meningeal or sinusogenous infection. Treatment is exclusively surgical (see Mastoiditis), leading in the vast majority of cases to a cure in the absence of involvement of the meninges and brain substance.