Cholesteatoma

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Classification according to ICD-10
H71 Cholesteatoma
H60.4 Ear canal cholesteatoma
H95.0 Recurrent cholesteatoma, mastoid cavity, after mastoidectomy
H66.2 Otitis media epitympanalis
ICD-10 online (WHO version 2019)

As cholesteatoma (synonym: Perlgeschwulst , onion tumor , otitis media epitympanalis ) of the ear is called a Einwucherung of multilayered keratinizing squamous into the middle ear with subsequent chronic purulent inflammation of the middle ear of mammals . The chronic otitis media associated with cholesteatoma is called chronic bone suppuration . The cholesteatoma is a progressive , destructive-inflammatory lesion . From a pathological point of view, the cholesteatoma shows the following characteristics: migration , increased cell proliferation , invasiveness, impaired cell differentiation , progression and tendency to relapse after surgical intervention.

In the healthy ear, the keratinizing squamous epithelium of the external auditory canal and the mucosal epithelium of the middle ear are completely separated from each other by the eardrum . If this barrier is broken, squamous cells can grow into the tympanic cavity . This leads to a spread of keratinizing squamous epithelium into the middle ear spaces.

Cholesteatomas can also develop outside the ear in the cranial cavity or wall of the skull.

Medical history

Joseph-Guichard Du Verney reported for the first time about a greasy mass in the temporal bone and called this "Stéatome". In 1829, Jean Cruveilhier described the mass in the temporal bone as tumeur perlée because of its macroscopic pearlescent appearance. The medical term "cholesteatoma" used to this day goes back to the German physiologist and comparative anatomist Johannes Müller , who counted it as a benign fatty tissue tumor.

In 1850, Joseph Toynbee described under the name molluscous tumor and later also sebeaceous tumor neoplasms in the ear, which Toynbee viewed as primary follicle tumors (tumors from epidermal scales delimited by a bellows) of the ear canal, which, however, resembled a cholesteatoma. He in turn saw their origin in the sebum glands.

As part of the operations for chronic suppuration of the mastoid process, it was Ernst von Bergmann who in 1899 asked for the posterior wall of the ear canal to be removed. He called this surgical step "radical surgery of the ear".

Pathogenesis

Physiologically, the tympanic cavity is lined with a single-layer epithelium made up of cubic (isoprismatic) to flat epithelial cells. Penetrate this tissue system keratinizing squamous one, so it will be a proliferation of keratinocytes of the squamous or develop these from embryonic Mesenchymresten these start with an altered growth behavior to proliferate and there is an onion-like bead of keratinizing squamous cells, which form the Cholesteatommatrix is the destruction of the surrounding tissue is the result.

The histologically different sections of the eardrum must be taken into account. The small pars flaccida or Shrapnell's membrane consists of only two layers; Towards the external auditory canal there is a multi-layered squamous epithelium, stratum cutaneum , while a single-layer squamous epithelium, stratum mucosum , is formed towards the tympanic cavity . Only the basal lamina lies between the two different cell layers . It is different with the larger pars tensa , here the two cell layers and the basal lamina are also formed, but two connective tissue fiber layers, stratum fibrosum or lamina propria , can also be distinguished. One of these fiber layers has a radial course, stratum radiatum, and the other is arranged in a circular manner, stratum circulare .

The histologically different structure leads, in the sense of a predilection point, to a more favorable occurrence of a primary cholesteatoma in the area of ​​the pars flaccida . Chronic tube ventilation disorders can lead to a retraction pocket in the pars flaccida . In such a retraction pocket, epithelial exfoliation occurs , which subsequently accumulates in the tympanic cavity and over time and due to changed cellular factors then form a cholesteatoma.

Indications of the changed conditions in comparison to the normal squamous epithelial layer of the eardrum are the detectable high concentrations of the growth factors Epidermal Growth Factor Receptor (EGFR) , Transforming growth factor, TGF-α , interleukin-1, Il-1 in the cholesteatome matrix . All of which indicate stronger growth activity of the keratinocytes. The plasminogen activator system, PAS and the matrix metalloproteinase system also play an important role. Tissue proliferation requires an exchange of substances, which in turn requires angiogenesis . Although such a new vessel formation u. a. also represents the prerequisite for physiological growth in the organism, but it also plays an important role in wound healing after tissue damage. The squamous epithelium of the cholesteatommatix cannot stimulate the formation of new vessels, but the surrounding perimatrix and its cellular components are of mesenchymal origin and as such are capable of inducing angioneogenesis.

Large atticcholesteatoma (arrow), left ear
Tympanic sinus cholesteatoma, left ear
Auditory canal polyp from granulation tissue in cholesteatoma
Cholesteatoma: squamous epithelium with horny lamellae and the perimatrix on the right side. Histology. HE staining .
Cholesteatoma: Pronounced chronic inflammatory reaction. Histology. HE staining.
Cholesteatoma: granulation tissue polyp. Histology. HE staining.

The Cholesteatomperimatrix must be distinguished from the Cholestatommatrix, this arises from the subepithelial connective tissue. Thus, from a pathohistological point of view, two essential layers can be represented on the cholesteatoma .

The cholestatom matrix is ​​the superficial epithelial layer with keratinizing squamous cells and cell detritus. It resembles the eardrum epithelium and the auditory canal epithelium, but without its appendages.

In contrast, the perimatrix represents the underlying connective tissue, it is responsible for the exchange of substances in the epithelium and is inflammatory activated. The perimatrix, i.e. the inflammation- activated connective tissue , histomorphologically consists of the following cells: fibroblasts , macrophages , lymphocytes , mastocytes , granulocytes , endothelia and the osteoclasts activated in the bone .

Depending on the inflammatory activity, their thickness and extent differ. If there is high inflammatory activity, the basement membrane between the matrix and the perimatrix may be interrupted in places. This accompanying inflammatory reaction leads, via the action of various cytokines, to inflammation-related activation and chemotaxis of the osteoclasts in the bone surrounding the cholesteatoma . This inflammation can spread to the bony parts of the middle ear and inner ear , which are then gradually destroyed.

Presumably due to the disturbed discharge of secretion and a bacterial superinfection - often with Pseudomonas aeruginosa - the ingrown epithelium leads to an increase in the inflammatory process.

Scheme of an unremarkable otoscopic finding. Right eardrum seen from the lateral side . Light gray pars flaccida of the eardrum and manubrium of the hammer (malleus) , dark gray pars tensa of the eardrum with light reflex, (here cone of light ), pink edge of the eardrum, anulus fibrocartilagineus

The cross-section of the cholesteatoma consists of snow-white, onion-skin-like, dead epithelial layers superimposed on one another. These are encased by keratinizing epithelium (matrix), which produces the epithelial layers, and a (mostly) inflammatory changed layer of mucous membrane (perimatrix), which is responsible for the destruction of the bone. There are also inflammatory and regressive changes.

Assuming an inflammatory process, one can divide the form of otitis media chronica , i.e. a chronic otitis media , into one of:

  • Chronic mucosal suppuration, including chronic mesotympanic otitis ; which means that most of the time the eustachian tubes cause inflammation in the middle ear . The central defect in the eardrum is characteristic of this disease. The edge of the eardrum, annulus fibrosus, is preserved everywhere.
  • Chronic suppuration, including chronic epitympanal otitis media ; this is a form of cholesteatoma. When looking at the eardrum with the otoscope , one typically finds a defect at the edge, and whitish granulations or red pseudopolyps in the defect.

In total, four theories are offered for the development of the acquired cholesteatoma, i.e. the primary and secondary cholesteatoma:

  • the immigration theory ; an eardrum defect allows peripheral keratinocytes of the multilayered squamous epithelium, stratum corneum, to grow into the tympanic cavity and thereby induces an inflammatory reaction. This inflammatory milieu creates a growth stimulus for the squamous epithelium.
  • the metaplasia theory ; Expressed cytokines lead to metaplasia of the epithelium in the monolayered squamous epithelium, stratum mucosum of the tympanic cavity.
  • the retraction or retraction pocket theory ; In this theory , the focus is initially on the mechanical effect of a negative pressure or underpressure in the tympanic cavity, caused by a (chronic) tube ventilation disorder. This promotes the formation of a retraction pocket and an accumulation of keratinocytes then forms the basis for the cholesteatoma that forms.
  • the theory of basal cell hypertrophy ; Keratinocytes can penetrate a patchy basal lamina under certain circumstances. In the connective tissue lamina propria or stratum fibrosum and thus penetrated into the subepithelial space, so-called inclusion cysts develop due to increasing cornification.

Classification

The classification of inflammatory middle ear cholesteatomas can be made from various points of view, primary , secondary or topographical anatomical. The most plausible form is that of the division according to the place of origin.

With regard to the origin or the place of origin, one can differentiate between three forms of cholesteatoma:

Primary cholesteatoma

There is no history of inflammation in primary cholesteatoma.

  • Retraction cholesteatoma: The origin of the cholesteatoma is the formation of a so-called retraction pocket in the area of ​​the pars flaccida (Shrapnell's membrane) of the eardrum in the uppermost part of the eardrum, presumably due to a chronic disturbance of tube ventilation . The cholesteatoma develops from the retraction pocket and initially spreads in the dome of the middle ear ( attic ); this is why this cholesteatoma is also called an attic cholesteatoma .
  • Immigration cholesteatoma: It arises from active ingrowth of epithelial cones into the Shrapnell's membrane.

Secondary cholesteatoma

Secondary cholesteatoma has a history of otitis media or (chronic) otitis media.

The secondary cholesteatoma arises on the basis of an inflammatory, deeply drawn in, wafer-thin (= atrophic) eardrum scar in the posterior part of the eardrum ( sinus tympanicus cholesteatoma ) or through a so-called middle ear atelectasis, in which the entire eardrum changes atrophy and attaches to the inner wall of the The middle ear is sucked in ( pars tensa cholesteatoma ). Due to inflammatory changes and the accumulation of whitish cholesteatome material, the retraction pockets often appear as perforations of the eardrum. Real marginal perforations after necrotizing otitis (e.g. in scarlet fever ) as the starting point for a cholesteatoma are now rather rare.

Congenital cholesteatoma

The rare congenital cholesteatoma arises from cell disintegration during the embryonic phase, more precisely from incompletely regressed mesenchymal tissue in the submucosa of the middle ear. In contrast to the other cholesteatomas, the congenital cholesteatoma develops behind an intact eardrum and (at least primarily) without contact with the eardrum.

Symptoms

In case of complications:

Findings and diagnosis

The diagnosis is made with the help of ear microscopy. The typical finding is a defect in the pars tensa or pars flaccida of the tympanic membrane with evidence of whitish-yellow scales or cell masses in the lesion . Polyps from granulation tissue are not uncommon . If you examine the eardrum through direct otoscopy , you can often see a perforation of the eardrum at the upper or rear edge.

Structure of a right middle ear. Looking at the eardrum and middle ear. The location of the tympanic cavity, (Cavum tympani) ( denoted by ph )

The cholesteatoma is usually located in the upper, rear part of the tympanic cavity, so that its detritus empties into the auditory canal through a perforation on the pars flaccida of the eardrum, more rarely also on the pars tensa but then at the edge near the fibrous ring, the anulus fibrocartilagineus .

Further examinations, diagnoses

Imaging procedures

Furthermore, computed tomography recordings (CT) produced. For this purpose, high-resolution spiral computed tomography has established itself as the method of first choice. With it it is possible to show the delicate morphological structures of the temporal bone region, a region that demands the highest demands on the spatial resolution and the image quality of CT images. High-resolution computed tomography (HR-CT ) enables the anatomical structures to be displayed in thin-layer technology and in the bone window, in the axial and coronal plane. The basis of a high-resolution temporal bone CT is the axial projection plane.

Axial CT; Cholesteatoma in the left external auditory canal (right in the picture). Computed tomography shows a small, soft tissue-dense erosion of the bone.
Coronares CT; Cholesteatoma in the left external auditory canal (right in the picture). Computed tomography shows a small, soft tissue-dense erosion of the bone.
Computed tomography series of a cholesteatoma (red in the picture) in the dome of the middle ear

As an alternative to the described CT diagnostics, the HASTE (half-Fourier acquisition single-shot turbo spin-echo) sequence in magnetic resonance tomography is a new, radiation-free method that has an extraordinarily high specificity, making it an important one , especially when it comes to questions about relapses Role play.

Before the introduction of CT, special X-rays according to Schüller and Stenvers were used for diagnosis. The X-ray image according to Artur Schüller (1874–1957), or image according to Schüller, is an imaging of the skull. The X-ray plate lies on the side of the ear and the central beam comes from the opposite side at an inclination angle of 20 to 30 ° from above onto the auditory canal of the ear near the plate. In this way, the mastoid process with the mastoid antrum , the outer and inner auditory canals projecting over one another and parts of the temporomandibular joint can be captured.

The X-ray according to Hendrik Willem Stenvers (1889–1973), briefly after Stenvers, is also an X-ray of the skull. The X-ray plate lies obliquely to the side - at a 45 ° frontotemporal angle - in front of the eye socket, orbit and the zygomatic bone . The central beam comes at an angle of 12 ° to 15 ° offset to the horizontal, the virtual right angle to the X-ray plate. With this setting, a longitudinal display of the petrous bone can be made possible.

Lightening ; a dark area on an X-ray that is projected onto the middle ear or the mastoid antrum are often associated with cholesteatoma. In the advanced course of a cholesteatoma, a large, rounded defect (as a dark area in the X-ray according to Schüller) in the area of ​​the mastoid antrum can be seen radiologically, and there are often signs of more severe sclerosis of the bone in the mastoid process. This is the radiological expression of a chronic mastoiditis caused by the inflammatory processes in the cholesteatoma.

Audiometric method

The audio audiogram can provide evidence of conductive hearing loss .

therapy

A cholesteatoma can only be removed through surgery. As a rule, systemic antibiotic therapy is carried out to reduce the inflammatory process and create better surgical conditions.

If the cholesteatoma can be completely removed during the operation, the eardrum and ossicles can be restored ( tympanoplasty ).

In the case of extensive cholesteatoma or significant destruction, a radical cavity must be created by a radical mastoidectomy . A common cavity is created from the auditory canal, the mastoid process cavity and the dome of the middle ear , which can be seen from the outside through the enlarged auditory canal opening and accessible for cleaning. In this case, too, the former middle ear can often be closed with a new eardrum and the hearing ability can thus be improved again (so-called flat tympanic membrane).

The primary goal of the surgical procedure is to clean up the destructive-inflammatory process in the mastoid and the tympanic cavity. The operative goal is the complete evacuation of the cholesteatoma. The aim is to improve or maintain hearing only secondarily , so that a two-stage operation such as a tympanoplasty is necessary.

surgery

The answer to the question of whether the posterior wall of the auditory canal and the mastoid antrum can be preserved or whether it has to be removed is crucial for surgical planning . When planning operational renovation, a basic distinction is made between an open and a closed approach. When overlapping the cholesteatoma on the mastoid , mastoid two procedures must be described:

  • the open approach or engl. canal wall down technique (CWDT) or
  • the closed approach of the engl. intact canal wall technique (ICWDT) mastoidectomy.

In the open approach, canal wall down technique (CWDT) with the creation of a so-called radical cavity , the posterior wall of the auditory canal, the lateral attic wall and successively the cholesteatoma are removed. The mastoid ( mastoid ) is drilled to sift Cholesteatomanteile located therein and to remove. A wound cavity is thus formed consisting of the dome of the middle ear (epitympanon), the drilled mastoid cavity and the auditory canal with removal of the posterior wall of the auditory canal. This wound or radical cavity is formed via an access behind the ear ( retroauricular ) or in the ear ( enaural ) through the surgical removal of the lateral dome wall and also the posterior bony wall of the auditory canal. In the case of an initially large wound cavity, an attempt is made to reduce it, for example by hammering in a muscle-periosteal tissue flap. A prerequisite for this, however, is a safe removal of all cholesteatoma foci.

With the closed procedure, intact canal wall technique (ICWDT), the posterior wall of the ear canal and thus its function is preserved or reconstructed. Either parts of the bony posterior wall of the auditory canal or elastic cartilage tissue of the tragus are used for reconstruction .

At the end of the operation, the surgical cavity is tamponized and removed or renewed after 5 to 7 days.

Aftercare

After a cholesteatoma operation, the operated patients have to undergo regular ENT examinations.

Risks and possible complications

literature

Web links

Commons : Cholesteatoma  - collection of images, videos and audio files

Individual evidence

  1. ^ Rudolf Probst; Gerhard Grevers; Heinrich Ivo: ear, nose and throat medicine. Georg Thieme, Stuttgart / New York 2000, ISBN 3-13-119031-0 , p. 243
  2. ↑ Traité de l'organe de l'ouïe, contenant la structure, les usages et les maladies de toutes les parties de l'oreille (in Latin: 1683, 1730; in French: 1718, 1731 (online) , 1783; in German : 1732)
  3. ↑ Jean Cruveillhier: Anatomie pathologique du corps humain, ou descriptions, avec figures lithographées et coloriées, des diverse alterations morbides dont le corps humain est susceptible. Baillière, Paris 1829
  4. ↑ Johannes Müller: About the finer structure and the forms of the pathological tumors. G. Reimer, Berlin 1838
  5. ↑ The London Medical Gazette, 1850, November and Medico-Surgical Transactions. Vol. 45. VII. Series
  6. ↑ Hermann Schwartze: Historical note on cholesteatoma of the temporal bone. Archive for Ear Medicine, December 5, 1901, Volume 54, Issue 1–2, p. 141
  7. ↑ Esther Schimanski: History of the tympanoplasty. 2004 (Dissertation, Ruhr University Bochum), p. 24, online
  8. ^ Karl-Heinz Vosteen: The development of ear, nose and throat medicine in the 19th century. On the history of society and ear, nose and throat medicine.
  9. ↑ H. Sudhoff, J. Bujia, A. Fisseler-Eckhoff, H. Hildmann: Functional characterization of the middle ear mucosa remains in cholesteatoma. 1994 negotiation report of the German Society for Ear, Nose and Throat Medicine, Head and Neck Surgery Volume 1994/2, 1994, pp. 126–127.
  10. ^ Günter Klöppel; Antonio Cardesa; Wolfgang Remmele; Hans H. Kreipe; Thomas Mentzel; Pierre Rudolph; Pieter Slootweg: Pathology. Volume 7, Springer, 2008, ISBN 3-540-72884-8 , p. 294
  11. ↑ Ralph Litschel: The plasminogen activator system and matrix metalloproteinase system in the cholesteatoma of the middle ear. 2002, (Dissertation, Ruhr University Bochum), p. 8 f., Online
  12. ↑ Michael Sand: Investigation of the expression of osteoclast-stimulating and osteoclast-differentiating factors in the cholesteatoma. 2003, (Dissertation, Ruhr University Bochum), online
  13. ↑ E. Steinbach; A. Pusalkar; H. Heumann: Cholesteatoma pathology and treatment. Adv Otorhinolaryngol (1988) 39: 94-106.
  14. ↑ Jörg Ebmeyer: Investigations on the osteoclastic bone loss in the cholesteatoma. 2002, (Dissertation, Ruhr University Bochum), online
  15. ↑ E. Kastenbauer; V. Schilling; J. Bujia: Current information on the etiology and pathogenesis of cholesteatoma - an overview. Laryngo-Rhino-Otol 1994; 73 (1): 2-6 doi: 10.1055 / s-2007-997071 .
  16. ↑ Holger Sudhoff; H. Hildmann: Current theories on the development of cholesteatoma. Springer, HNO 2003 51: 71-83 doi: 10.1007 / s00106-002-0770-1
  17. ↑ Florian C. Uecker: Ear, nose and throat medicine in questions and answers. Urban & Fischer, Munich 2006, ISBN 978-3-437-41023-9 , pp. 28-29
  18. ↑ Lennart Edfeldt: Middle Ear Cholesteatoma. Surgical Treatment, Follow-up and Hearing Restoration. Acta Universitatis Upsaliensis Uppsala 2013, Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine 905 , ISSN  1651-6206 , p. 14
  19. ↑ W. Becker; HH Naumann; CR Pfaltz: Ear, Nose and Throat Medicine. Short textbook with atlas part differential diagnostic tables. Georg Thieme, Stuttgart / New York 1986, ISBN 3-13-583003-9 , p. 98 f.
  20. ↑ J. Nevouxa; M. Lenoir, G. Roger; et al .: Childhood cholesteatoma. European Annals of Otorhinolaryngology, Head and Neck diseases (2010) 127, 143-150
  21. ↑ The eardrum is folded in with a fibrous ring, the annulus fibrocartilagineus, in a groove in the surrounding bone, sulcus tympanicus of the auditory canal
  22. ↑ An HR-CT of the petrous bone requires the following technical prerequisites due to the large differences in density, the small size and the complicated morphology of the anatomical structures: minimum slice thickness of 0.5–1 mm; a large zoom factor; a high resolution algorithm; the use of a sufficiently large matrix (512 × 512); a wide bone window; Examination in two planes or the creation of a secondary reconstruction with multi-line computer tomography devices; Odo Köster: Computed tomography of the petrous bone. Thieme, Stuttgart / New York 2004 ISBN 3-13-721402-5
  23. ↑ Radiopaedia
  24. ↑ A. Alvo, C. Garrido, A. Salas, G. Miranda, CE Stott, PH Delano: Use of Non-Echo-Planar Diffusion-Weighted MR Imaging for the Detection of Cholesteatomas in High-Risk Tympanic Retraction Pockets. In: AJNR. American journal of neuroradiology. [electronic publication before printing] May 2014, ISSN  1936-959X . doi: 10.3174 / ajnr.A3952 . PMID 24812017
  25. ↑ AT Ilıca, Y. Hıdır, N. Bulakbaşı, B. Satar, I. Güvenç, HH Arslan, N. Imre N .: HASTE diffusion-weighted MRI for the reliable detection of cholesteatoma. In: Diagnostic and interventional radiology (Ankara, Turkey). Volume 18, Number 2, 2012 Mar-Apr, pp. 153-158, ISSN  1305-3612 . doi: 10.4261 / 1305-3825.DIR.4246-11.3 . PMID 21960134
  26. ^ Hans-Georg Boenninghaus; Thomas Lenarz: ENT. 12th edition, Springer, Berlin / Heidelberg / New York, 2004, ISBN 3-540-21969-2 , pp. 58–59
  27. ^ Francis A. Burgener; Martti Kormano: Radiographic differential diagnosis. From findings to diagnosis. Thieme, Stuttgart / New York 1988, ISBN 3-13-709801-7 , p. 125
  28. ↑ Jürgen Theissing: ENT operation theory. With all important interventions. Thieme, Stuttgart / New York 1996, ISBN 3-13-463703-0 , p. 357
  29. ^ Rudolf Probst; Gerhard Grvers; Heinrich Ivo: ear, nose and throat medicine. Georg Thieme, Stuttgart 2000, ISBN 3-13-119031-0 , p. 247
  30. ↑ Representation of a CWDT.
  31. ↑ Representation of an ICWDT.
  32. ↑ Stefanie Czibor: The need for post-treatment of the radical cavity. 2001, (Dissertation, Ruhr University Bochum) (PDF; 339 kB)
  33. ↑ H. Ganz; V. Jahnke: ear, nose and throat medicine. Walter de Gruyter, Berlin / New York 1996, ISBN 3-11-014742-4 , p. 67 f.