Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4408_Библиотеки_им_академика_М_И_Перельмана
.pdf
402
https://t.me/medicina_free
Fig. 41.6 This gure shows, respectively, a right ear with an atical retraction and a left ear with atical and posterior retraction with ossicular erosion, both with the accumulation of epithelial debris in the retraction (cholesteatoma).
I. Canali et al.
alteration in the PF was the erosion of the scutum, whereas,
in PQ, it was the erosion of the incus, suggesting that PQ
retractions would be more frequently associated with conductive hearing loss. Another study by our group has shown
that incus erosion is the alteration associated with larger air–
bone gaps (ABGs) in posterior mesotympanic cholesteatomas when compared with posterior epitympanic [35].
Regarding the audiometric pattern of these ears, few studies correlated the degree of retraction and the severity of
hearing loss. Cassano and Cassano evaluated 45 ears of children with varying degrees of TM retraction severity, based
on the Sadé's classication. They observed that in grades I
and II, the average hearing threshold was less than 10 dB HL
in 68.7% of ears and greater than 20 dB HL in 18.7%. In
grade III, the average hearing threshold was less than 10 dB
HL in 29.1% and greater than 20 dB HL in 25%. In those
with grade IV, no one had an average threshold of less than
10 dB, and the threshold was greater than 20 dB HL in 80%
[36].
In our study, we also demonstrated that 72% of the ears
had an ABG lower than 20 dB HL, and only 5% had an ABG
greater than 40 dB HL.These ndings meet those in the literature [36, 37].
We found that the pure tone average (PTA) ABG median
was greater when the posterior quadrants were involved,
exclusively or not. This can be explained by the ossicular
chain involvement in this region. It is well known that chronic
otitis media can compromise the TM's effectiveness and/or
the ossicular chain's vibratory pattern. Therefore, a variable
degree of conductive hearing loss is almost omnipresent in
these patients. The pathogenesis of hearing loss is related to
several factors, including partial or total vibratory TM surface loss, and ossicular chain erosion or xation.
We observed a signicant correlation between the severity of retraction and the worsening of AC and ABG threshold, only for PT.These ndings indicate that ABG measure
is not inuenced by even more severe PF involvement.
However, the severity of ossicular erosion in the posterior
quadrants was predictive of an increase in the ABG and
worsening of an AC.
The retractions were systematically classied in the literature, according to the region of TM affected by PF and
PT.In our study, we analyzed the behavior of retractions in
all areas of TM, and we observed that the majority of retractions had an association with affected regions. Jesic etal.
showed that the combination of PF and PT retractions in
the same ear was a predictor of a long incus process and
stapes superstructure erosion [38]. However, the ABG's
PTA was not a predictor of ossicular chain erosion. This
corroborates with the existing data, which shows no correlation between hearing thresholds and the stages of retraction [36–38].
Only a few studies have investigated the magnitude of
hearing loss in specic cases of myringostapediopexy
(retraction limited to the posterosuperior region, in which
erosion of the long process of the incus and xation of the
41 Tympanic Membrane Retractions: Pathophysiology, Classication, andManagement
https://t.me/medicina_free
In a study of our group, 47% of the samples were children, and we evaluated the differences between children and
adults based on the location of the retractions, their severity,
and the degree of the associated air–bone gap (ABG). We
found a higher prevalence of retractions involving the PT in
children, isolated or not, and of isolated PF retractions in
adults. These ndings are consistent with the literature,
wherein most retractions located in the PT in pediatric
patients, which is also the most frequent site of acquired cholesteatomas in the current population. PF retractions, on the
other hand, seem to be more prevalent in adults [33, 37]. In a
previous study by our group, we also found that acquired
cholesteatoma exhibited the same pattern with respect to
their location; posterior mesotympanics were more prevalent
in children, and posterior epitympanic (attical) in adults [42,
43]. Likewise, we had already observed in another previous
study, involving patients with ME cholesteatoma, that when
present, both moderate and severe TM retraction and cholesteatoma in the contralateral ear tend to follow the same path-
Fig. 41.7 A right ear with myringostapediopexy
way of cholesteatoma formation in the main ear. These
ndings reafrm the important role played by TM retractions
affected tympanic segment to the stapes head occurs, regardless of cholesteatoma formation—Fig. 41.7) [39]. A study by
our research group evaluated conductive hearing loss in this
condition [26]. The tritonal average of the ABG was 18.46dB
HL.Fifty-three percent of the patients had an ABG equal to
or lower than 25dB HL at all frequencies. The analysis of
tritonal PTA revealed that 85% of all ears with natural myringostapediopexy had conductive hearing loss equal to or
lower than 25dB HL.In the study done by Mills, 31 ears
presented the PTA of the ABG were less than 20dB HL in
77% of the ears, and only 3.2% had an ABG greater than
40dB HL [40]. These ndings suggest that these cases are
similar to a type III tympanoplasty in relation to hearing loss.
The signicant repercussions of the degree of hearing loss
in these cases, especially of the ABG, depend on the decision
whether the patient will benet from early intervention or
not.
Few studies in the literature have compared the degree of
severity of retractions in children and adults. TM retraction
may be associated with a conductive hearing loss, especially
when the posterosuperior quadrant is compromised [1, 41].
The difference in prevalence of cholesteatoma in children
and adults has already been demonstrated in previous studies, with the posterior mesotympanic cholesteatomas being
more prevalent in the rst group [42, 43]. The severity of
hearing loss is comparable in these two age groups as demonstrated in studies on cholesteatomas [42, 43]. However,
there are no studies that compare the severity of hearing loss
in children and adults in the former stages of cholesteatomas
such as in tympanic retractions.
in the pathogenesis of cholesteatoma [21].
We also observed that, as the age of the patients increases,
the prevalence of attical (PF) retractions also increases progressively, and the opposite occurs for the prevalence of
retractions located in the PQs (PT), irrespective of their association with attical impairment, whose frequency decreases
as the age increases. The explanation for such ndings would
be that, in childhood, for reasons mainly related to the dysfunction of the auditory tube and the occurrence of gas
exchange through the hyperplastic mucosa of the ME, the
maintenance of the negative pressure sustained in the auditory cleft would be the main cause of TM retraction and its
preferred location in PT.However, as the retraction of the PT
persists, the medialization of the manubrium of the malleus,
hyperplasia of the mucosa of the ME, and the presence of the
secretion plugs could determine the obliteration of the tympanic isthmus, contributing to the epitympanic dysventilation syndrome and, thus, resulting in the concomitant
occurrence of attical retraction. The changes that lead to PT
retraction would be easier to resolve over time. The functioning of the auditory tube can improve as the child grows.
Likewise, recurrent otitis media and upper airway infections
are also becoming less frequent, consequently reducing the
inammatory processes in the ME mucosa. However, the
compartmentalization of the epitympanum, with persistence
of the isthmus blockage and appearance of brosis, is not a
process that would resolve so easily on its own, thereby
persisting over the years. This could explain the higher prevalence of isolated PF retractions and, consequently, posterior
epitympanic cholesteatomas, in adults [1, 44].
403
404
https://t.me/medicina_free
I. Canali et al.
We also observed that there was no difference between
children and adults regarding the severity of isolated retractions of both PT and PF, demonstrating that the severity of
the retraction can already be established at earlier ages. In
our study, there was no difference in the ABG size between
children and adults, regardless of the area of TM involvement. The management of the moderate and severe tympanic
membrane retractions will be discussed in a separated
chapter.
Management ofRetractions
Managing TM retractions is one of the most exciting topics
for otologists. Many controversies associated with this condition are based on the difculty in understanding the pathophysiological characteristics, which make some TM
retractions remains stable in the long term and others easily
destabilize, possibly evolving with epithelial ow.
The difculty is based on the fact that most patients are
asymptomatic and hearing loss is, in most cases, small,
even in cases where there is ossicular chain erosion [31].
The great dilemma is usually: should the surgical intervention be early, being prophylactic, or should the intervention be postponed until the development of cholesteatoma
or hearing loss? The answers to this question are
divergent.
Behavior ofRetractions
The natural history of TM retractions is that they can follow
one of four destinations: (1) resolution, (2) stabilization, (3)
perforation, and (4) keratin accumulation with cholesteatoma formation.
Mechanisms responsible for the progression of retractions continue to be debated. Tubal dysfunction, impairing
middle ear ventilation, remains an important factor in the
evolution to cholesteatoma. Primary acquired cholesteatoma
tend to be the most frequent and originate, in most cases, as
a result of the progression of a pars accida (PF) retraction
pocket medially and depth toward the epitympanum, or of a
retraction pocket of the posterosuperior quadrant toward the
mesotympanum [11, 20, 42, 43, 45].
Experiments in guinea pigs, conducted by Chole et al.,
demonstrate the development of cholesteatoma from TM
retractions. In their studies, they observed that the ET cauterization in gerbils resulted in cholesteatoma in 40–75% of the
ears after 16 weeks of obstruction [46]. They also demonstrated that infection by Pseudomonas Aeruginosa made
cholesteatoma present a more aggressive behavior, with
faster growth, when compared to noninfected ones. The sustained inammatory process of the middle ear (ME) mucosa
leads to a change in gas transmission blocking the auditory
cleft, adding to the processes mentioned above [47].
We believe, therefore, that the inammatory factors that
break the stability of the retractions are essential for this
evolution.
There is a dearth of literature on the natural history of
retractions and their evolution. This can be attributed to the
fact that monitoring these ears is time-intensive, and there is
a consequent loss of patients to follow-up. Although there
are many studies on retractions, they are not subject to comparisons, since the inclusion criteria are not the same and the
classications are different, and, therefore, are not in agreement with one other.
Management
The progression to cholesteatoma and hearing loss are two
main concerns regarding retraction pockets and should and
must be taken into account when dening their management
[41, 44]. Also we have to take in account the presence of
recurrent infections, the state of the contralateral ear, and the
ET function.
Different managements have been proposed in the treatments of retractions, including: observational treatment,
insertion of ventilation tubes, excision of the retraction
pocket associated with tympanoplasty with fascia and/or
cartilage graft, and tympanomastoidectomy. Ossiculoplasty
may be necessary, depending on the status of the ossicular
chain and hearing loss. When observational treatment is
indicated, careful surveillance must be performed, with
serial clinical and audiometric evaluations every six
months [25].
In a meta-analysis, conducted by Nankivell and Pothier,
on the treatment of retraction pockets, 71 patients were
included [48]. No statistically signicant benet was
obtained from cartilage tympanoplasty, on observational
conduct, in relation to disease progression and hearing loss.
They also observed that there was no benet from the insertion of ventilation tube associated with cartilage graft, when
analyzing the hearing improvement [48].
Despite all the controversies regarding the management
of retractions, there are two situations in which surgical
intervention is somehow indicated, without question: when
there is keratin in the retraction pocket (cholesteatoma) and
when there is signicant hearing loss [14, 33].
It is needed to correlate the severity of otoscopic ndings
with the degree of conductive hearing loss in order to dene
the need for an early surgical intervention for each case.
Whether the surgical intervention must be early, prophylactic,
or should be postponed until the development of epithelial
accumulation (cholesteatoma) or signicant hearing loss is
debatable. Perhaps this question is even more pertinent in the
41 Tympanic Membrane Retractions: Pathophysiology, Classication, andManagement
https://t.me/medicina_free
405
pediatric population, since children tend to have a higher
prevalence of recurrent infections and, probably, longer periods of instability and otorrhea. In addition, identifying the
location of the retraction (preferably involving the posterior
quadrant) precisely on the ossicular chain would help determine the degree of risk of hearing loss, based on our previous
study, or development of later mesotympanic cholesteatomas, which are generally associated with greater hearing
impairment and more frequent recurrences [42, 43]. On the
other hand, mild tympanic retraction can be solved without
surgery; especially in the case of children, in whom it evolves
to resolution in 35% of affected ears [36, 49].
The signicant repercussions of the degree of hearing loss
in these cases, especially of the air–bone gap (ABG), depend
on the decision whether the patient will benet from early
intervention or not. As described in the literature, we know
that stapes superstructure erosion is one of the main predictors of poor surgical results; therefore, the probability of a
favorable postsurgical outcome is uncertain in patients with
small ABG.
A review of studies on surgical indication and hearing
results of ossiculoplasty revealed that the minimum preoperative ABG required ranges from 24 to 27 dB HL. The
American Academy of Otolaryngology Committee on
Hearing denes treatment success as conductive hearing loss
equal to or lower than 20 dB HL [50]. The rates of treatment
success ranged from 47% to 86.9% with total ossicular
reconstruction prosthesis (TORP) and from 50% to 84.6%
with partial ossicular reconstruction prosthesis (PORP)
[51–53].
The literature often shows better audiometric results with
PORP than with TORP.However, a meta-analysis comparing the two groups showed better outcomes with TORP
[31]. All studies agree that the presence of stapes determines
a better prognosis in the results of ossiculoplasty [53].
Although the stapes superstructure does not provide additional audiometric gain, it promotes better stabilization and
xation of the prosthesis [54]. Thus, reconstruction of the
ossicular chain, and tympanoplasty, would not be justied
in retraction cases with an ABG lower than 25 dB HL, especially in patients with stapes erosion. However, factors such
as the presence or progression to cholesteatoma and the frequency of ear infections must be considered when monitoring therapies.
It is important to emphasize that, in the management,
especially of the attic retractions, the reestablishment of the
ventilation pathways to the epitympanum is important in
order to promote direct ventilation to the attic region and prevent the recurrence of the retraction pocket and formation of
cholesteatoma [11, 20].
Finally, we believe that management should be individualized for each patient, considering not only the topographical and momentary description of the severity of the
retraction but also evaluating the hearing in each case, as
well as the degree of stability of these ears, and the state of
the contralateral ear.
References
1. Alper C, Olszewska E.Assessment and management of retraction
pockets. Otolaryngologia Polska. 2017;71(1):1–21.
2. Seibert JW, Danner CJ. Eustachian tube function and the middle
ear. Otolaryngol Clin N Am. 2006;39(6):1221–35.
3. Bluestone CD.Eustachian tube function: physiology, pathophysiology, and role of allergy in pathogenesis of otitis media. J Allergy
Clin Immunol. 1983;72(3):242–51.
4. Doyle WJ, Seroky JT, Alper CM. Gas exchange across the middle ear mucosa in monkeys. Estimation of exchange rate. Arch
Otolaryngol Head Neck Surg. 1995;121(8):887–92.
5. Paparella MM, etal. Cellular events involved in middle ear uid
production. Ann OtolRhinolLaryngol. 1970;79(4):766–79.
6. Magnuson B, Falk B. Diagnosis and management of eustachian
tube malfunction. Otolaryngol Clin N Am. 1984;17(4):659–71.
7. Asawapittayanont P, et al. Tubal function tests with optional
myringotomy detect Eustachian tube closing failure in acquired
pars accida retraction cholesteatoma. Auris Nasus Larynx.
2017;44(1):65–9.
8. Levy D, etal. Direct demonstration of gas diffusion into the middle
ear. Acta Oto-Laryngol. 1995;115(2):276–8.
9. Bunne M, et al. Variability of Eustachian tube function: comparison of ears with retraction disease and normal middle ears.
Laryngoscope. 2000;110(8):1389–95.
10. Licameli GR.The eustachian tube. Update on anatomy, development, and function. Otolaryngol Clin N Am. 2002;35(4):803–9.
11. Marchioni D, etal. Prevalence of ventilation blockages in patients
affected by attic pathology: a case-control study. Laryngoscope.
2013b;123(11):2845–53.
12. Palva T, Northrop C, Ramsay H.Aeration and drainage pathways of
Prussak’s space. Int J PediatrOtorhinolaryngol. 2001;57(1):55–65.
13. Palva T, Ramsay H.Incudal folds and epitympanic aeration. Am J
Otolaryngol. 1996;17(5):700–8.
14. Costa S, etal. Otitesmédias: aspectosgerais. In: Cruz O, Costa S.
(org.). Otologiaclínica e cirúrgica. Rio de Janeiro: Revinter, 2000.
pp.137–161.
15. Maniu A, etal. Anatomical variants of tympanic compartments and
their aeration pathways involved in the pathogenesis of middle ear
inammatory disease. Clujul Med. 2013;86(4):352–6.
16. Marchioni D, etal. The contribution of selective dysventilation to
attical middle ear pathology. Med Hypotheses. 2011;77(1):116–20.
17. Palva T, Ramsay H.Epitympanic diaphragm in the new-born. Int J
PediatrOtorhinolaryngol. 1998;43(3):261–9.
18. Marchioni D, etal. Selective epitympanic dysventilation syndrome.
Laryngoscope. 2010b;120(5):1028–33.
19. Marchioni D, etal. Endoscopic evaluation of middle ear ventilation
route blockage. Am J Otolaryngol. 2010a;31(6):453–66.
20. Marchioni D, etal. Endoscopic anatomy and ventilation of the epitympanum. Otolaryngol Clin N Am. 2013a;46(2):165–78.
21. Rosito LPS, etal. The role of tympanic membrane retractions in cholesteatoma pathogenesis. BioMed Res Int. 2018;2018. (online edition)
22. Shunyu NB, et al. Histological and immunohistochemical study
of pars tensa retraction pocket. Otolaryngol Head Neck Surg.
2011;145(4):628–34.
23. Urík M, etal. Histological analysis of retraction pocket pars tensa
of tympanic membrane in children. Int J PediatrOtorhinolaryngol.
2016;86:213–7.
24. Sade J, Berco E. Atelectasis and secretory otitis media. Ann
OtolRrhinolLaryngol. 1976;85(2 Suppl 25 Pt 2):66–72.
406
https://t.me/medicina_free
I. Canali et al.
25. Alzahrani M, Saliba I. Tympanic membrane retraction pocket
staging: is it worthwhile? Eur Arch Oto-Rhino-Laryngol.
2014;271(6):1361–8.
26. Schmidt B, etal. Myringostapediopexy: is it a natural type III tympanoplasty? OtolNeurotol. 2013;34(1):79–82.
27. Tos M, Stangerup SE, Larsen P. Dynamics of eardrum changes
following secretory otitis. A prospective study. Arch Otolaryngol
Head Neck Surg. 1987;113(4):380–5.
28. Charachon R. Classication of retraction pockets. Rev
LaryngolOtolRhinol. 1988;109:205–7.
29. Sade J, Avraham S, Brown M.Atelectasis, retraction pockets and
cholesteatoma. Acta Oto-Laryngol. 1981;92(5–6):501–12.
30. Bours AF, Decat M, Gersdorff M. Our classication of tympanic retraction pockets. Acta Oto-Rhino-LaryngolBelg.
1998;52(1):25–8.
31. Dornhoffer JL.Surgical management of the atelectatic ear. Am J
Otol. 2000;21(3):315–21.
32. Borgstein J, etal. The Erasmus atelectasis classication: proposal
of a new classication for atelectasis of the middle ear in children.
Laryngoscope. 2007;117(7):1255–9.
33. James AL, et al. Tympanic membrane retraction: an endoscopic evaluation of staging systems. Laryngoscope.
2012;122(5):1115–20.
34. Canali I, etal. Assessment of Eustachian tube function in patients
with tympanic membrane retraction and in normal subjects. Braz J
Otorhinolaryngol. 2017;83(1):50–8.
35. Rosito LPS, etal. Hearing impairment in children and adults with
acquired middle ear cholesteatoma: audiometric comparison of 385
ears. OtolNeurotol. 2015;36(8):1297–300.
36. Cassano M, Cassano P. Retraction pockets of pars tensa in
pediatric patients: clinical evolution and treatment. Int J
PediatrOtorhinolaryngol. 2010;74(2):178–82.
37. Borgstein J, Gerritsma TV, Bruce IA.Erosion of the incus in pediatric posterior tympanic membrane retraction pockets without cholesteatoma. Int J PediatrOtorhinolaryngol. 2008;72(9):1419–23.
38. Jesic SD, et al. Pars tensa retractions without cholesteatoma in
children: predictors for ossicular chain destruction, air conduction thresholds, and postoperative retractions. OtolNeurotol.
2014;35(6):997–1002.
39. Monsell EM. New and revised reporting guidelines from the
Committee on Hearing and Equilibrium. American Academy
of Otolaryngology-Head and Neck Surgery Foundation, Inc.
Otolaryngol Head Neck Surg. 1995;113(3):176–8.
40. Mills RP. Management of retraction pockets of the pars tensa. J
LaryngolOtol. 1991;105(7):525–8.
41. Canali I, et al. Audiometric pattern in moderate and
severe tympanic membrane retraction. OtolNeurotol.
2021a;42(6):e716–23.
42. Rosito LPS, etal. Cholesteatoma growth patterns: are there audiometric differences between posterior epitympanic and posterior
mesotympanic cholesteatoma? Eur Arch Oto-Rhino-Laryngol.
2016a;273(10):3093–9.
43. Rosito LS, et al. Classication of cholesteatoma according to growth patterns. JAMA Otolaryngol Head Neck Surg.
2016b;142(2):168–72.
44. Canali I, etal. Critical analysis of moderate and severe retractions
in the pars tensa and pars accida of the tympanic membrane. Braz
J Otorhinolaryngol. 2023;89:114–21.
45. Jackler RK, Santa Maria PL, Varsak YK, Nguyen A, Blevins NH.
A new theory on the pathogenesis of acquired cholesteatoma:
Mucosal traction. Laryngoscope. 2015;125:S1–S14.
46. Wolfman DE, Chloe RA. Experimental retraction pocket cholesteatoma. Annals of Otology, Rhinology Laryngology.
1986;95:639–44.
47. Kania R, Portier F, Lecain E, Marcusohn Y, Ar A, Herman P, Huy
PTB. Experimental model for investigating trans mucosal gas
exchanges in the middle ear of the rat. Acta Oto Laryngologica.
2004;124:408–10.
48. Nankivell PC, Pothier DD. Surgery for tympanic membrane
retraction pockets. Cochrane Database of Systematic Reviews.
2010;7:CD007943.
49. Cutajar J, Nowghani M, Tulsidas Mahtani B, Hamilton J. The
Natural History of Asymptomatic Deep Pars Tensa Retraction. The
journal of internacional advanced otology. 2018;14:10–4.
50. Iñiguez-Cuadra R, etal. Type III tympanoplasty with titanium total
ossicular replacement prosthesis: anatomic and functional results.
OtolNeurotol. 2010;31(3):409–14.
51. Fong JCW, et al. Titanium versus autograft ossiculoplasty. Acta
Otolaryngol. 2010;130(5):554–8.
52. Vincent R, et al. Ossiculoplasty in intact stapes and malleus
patients: a comparison of PORPs versus TORPs with malleus relocation and silastic banding techniques. OtolNeurotol.
2011;32(4):616–25.
53. Yu H, et al. PORP vs. TORP: a meta-analysis. Eur Arch
Otorhinolaryngol. 2013;270(12):3005–17.
54. Alaani A, Raut VV.Kurz titanium prosthesis ossiculoplasty-followup-statistical analysis of factors affecting one ear hearing results.
Auris Nasus Larynx. 2010;37(2):150–4.
Temporal Bone Cholesteatoma: TheFull
https://t.me/medicina_free
Picture
SadySelaimenda Costa, LeticiaPetersenSchimdtRosito,
MauricioNoschangLopesda Silva,
andFábioAndréSelaimen
42
Introduction
Middle ear cholesteatomas are one of the most intriguing
otologic diseases, and their treatment demands a meticulous
and complete analysis of many different facets. As a rule, the
main discussion always narrows down (mistakenly) to the
surgical technique. Over the years, countless congresses,
symposiums, and meetings have been organized on ve continents with the aim of discussing advances in the understanding of this disease. As we mentioned earlier, the focus
of these debates emphasizes the following hierarchy:
1. THE SURGICAL TECHNIQUE or how do we do it?
2. THE SURGICAL TIMING or when to do it?
3. THE SURGICAL REATIONALE or why do we do it?
4. WHAT DOES ORIENT OUR DECISIONS or the
theoretical basis!
We believe that the best and most complete way to
approach this condition is to reverse this order. Thus, in our
opinion, the priority should be rearranged as follows:
1. WHAT DOES ORIENT OUR DECISIONS or the
theoretical basis!
2. THE SURGICAL REATIONALE or why do we do it?
3. THE SURGICAL TIMING or when to do it?
4. THE SURGICAL TECHNIQUE or how do we do it?
Thus, as at the base of a pyramid, the broad theoretical
base will support our therapeutic decisions, dening more
securely why, when, and how to treat. Not infrequently and
in very special situations, surgery may not be the best option
at a given time. Likewise, it is necessary to better and more
thoroughly understand the natural history of the disease and
its pathogenesis (masterfully dened by MM Paparella as
the “journey between the etiology and the established
pathology”). This is the best way to propose a set of rational
strategic actions throughout the process that may abort its
evolution and the potential risks of complications. We
always like to paraphrase the famous British neurosurgeon
Henry Marsh:
It is often said it takes three months to learn how to do an opera-
tion, three years to learn when to do it, and 30 years to learn
when not to do it.
S. S. da Costa (*)
School of Medicine- Federal University of Rio Grande do Sul,
Porto Alegre, Brazil
Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
International Hearing Foundation, Minneapolis, MN, USA
American Academy of Otolaryngology—Head and Neck Surgery,
Alexandria, VA, USA
L. P. S. Rosito
School of Medicine- Federal University of Rio Grande do Sul,
Porto Alegre, Brazil
Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
M. N. L. da Silva · F. A. Selaimen
Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
M. V. Goycoolea et al. (eds.), Textbook of Otitis Media, https://doi.org/10.1007/978-3-031-40949-3_42
The Expression
The German anatomist Johannes Mueller used the word
“cholesteatoma” for the rst time, in 1838 [1]. The root of
this word means chole (from cholesterol); steato from fatty
tissue), and oma (ending meaning tumor in biology), that is,
a tumor in which fatty tissue and cholesterol crystals are
present. Etymologically, this term is completely incorrect
since cholesteatomas originates from the keratinized stratied squamous epithelium of the tympanic membrane (TM)
and/or external auditory canal (EAC) and does not have cholesterol crystals or fat in its composition and, in addition to
its tumor nature being largely debatable.
407
408
https://t.me/medicina_free
Fig. 42.2 Digitized image of slide, with cross section of the cholesteatoma, stained with hematoxylin-eosin. We can see its three constituent
parts: (a)—perimatrix, (b)—matrix, and (c)—cystic content
Fig. 42.1 Human temporal section (left ear) showing a middle ear cholesteatoma lling the posterior mesotympanum. (* cholesteatoma;
black arrow: facial nerve; EAC external auditory canal, AM anterior
mesotympanum)
Other names have been suggested through the years such
as pearly tumor, by Cruveilhier (1829), margaritoma (Craigie
1891), epidermal cholesteatoma (Cushing 1922), epider-
moid by Critchley and Ferguson (1928), and keratoma by
Shuknecht, in 1974 (apud [1]). All these terms, although
more adequate and descriptive, have not become popular in
the medical literature and the word cholesteatoma denitely
has been enshrined among otologists.
The Denition
Friedmann dened cholesteatomas in 1959 [2], as cystic
structures covered by stratied squamous epithelium, resting
on a brous stroma of variable thickness, which may contain
some elements of the original mucous lining.
More simply, Schuknecht, in 1974 [3], denes them as
accumulation of exfoliated keratin inside the middle ear or
S. S. da Costa et al.
any pneumatized area of the temporal bone, arising from a
keratinized stratied squamous epithelium.
Ferlito etal. [4] describes the cholesteatoma as an epidermoid cyst, of independent and progressive growth, with
destruction of the adjacent tissues, especially the bone tissue,
with a tendency to recur (Fig.42.1).
Informally, we tend to simplify by referring to cholesteatomas as “skin in the wrong place!” Macroscopically, it is a
round or oval cystic lesion with variable shape and size; histologically, it may be broken into three main components: (1)
the matrix—keratinized stratied squamous epithelium; (2)
the perimatrix—a rich network of connective tissue, collagen bers, and inammatory cells; and (3) the cystic content—keratin and epithelial rests (Fig.42.2).
Epidemiology andRisk Factors
It is estimated that over 20 million people worldwide are
aficted with chronic otitis media (COM). Of these, onefourth (about ve million) have a cholesteatoma [5], although
the overall number of cases of acquired cholesteatoma seems
to be in decline [6, 7]. The annual incidence of cholesteatoma is reported as 3 per 100,000 in children and 9.2 per
100,000 adults. Males slightly outnumber females in a ratio
of 1.4:1, and cholesteatomas that present in the middle ear
are more frequently found in persons younger than 50years
of age [8, 9]. Caucasian persons show the highest prevalence,
but cholesteatoma is infrequently found in Inuit, Native
American, and Asian populations [10]. Several reports
reviewed by Jennings etal. evaluated familial clustering and
inheritability of cholesteatoma and found that incomplete
penetrance exists and may depend on a combination of environmental and genetic factors for the formation of an
acquired cholesteatoma. Jennings also states that evidence
from syndromic cases suggests genes controlling ear morphology may be risk factors for congenital or acquired cholesteatoma formation [11]. Syndromes where a diagnosis of
cholesteatoma has been reported nding include Turner syndrome [12–14], Treacher Collins syndrome[15], Down syndrome [16–19], and focal dermal hypoplasia [20]. Numerous
reports of patients presenting with cleft palate and cholesteatoma [21–27] have been presented with the rate of incidence
approaching 6% in that population [26]. When compared to
children who did not develop a cleft palate, those with a cleft
palate face a 100–200 times greater likelihood of developing
a cholesteatoma [22, 26]. Additionally, a link between allergic rhinitis and the development of cholesteatoma was
recently discovered in that patients with allergic rhinitis presented with a signicantly lower 10-year cholesteatoma
disease- free rate [28].
42 Temporal Bone Cholesteatoma: TheFull Picture
https://t.me/medicina_free
409
At the Chronic Otitis Media Outpatient Clinic at Hospital
de Clínicas de Porto Alegre (AOMC-HCPA), 2603 patients
were diagnosed with COM in follow-up. Of those, 638
(24.51%) presented cholesteatoma, which was bilateral in
17.1%. The mean age was 34, 49years, and 53.5% were
female. Also, most patients were 18years or older (63.8%).
Concerning the cholesteatoma growth patterns, the anterior
epitympanic was 1.9%, the posterior epitympanic (PEC) was
32.9%, the posterior mesotympanic (PMC) corresponded to
33.7%, two-route cholesteatoma was 14.8%, and open cholesteatoma (or indeterminate) was 16.7%.
Classication
Cholesteatomas are classied as congenital or acquired. The
former can be found in ve temporal bone regions; in turn,
the acquired can be subdivided into primary or secondary
(Table42.1).
Congenital cholesteatomas (CCs) were dened by
Derlacki and Clemis [29] as a conglomerate of epithelial
remnants found in ears with intact tympanic membranes and
usually without a previous history of infections. According
to Valvassori [30], they can be found in four regions of the
temporal bone: tympanic-mastoid, petrous apex, cerebellopontine angle, and jugular foramen. There is still a fth location, described by Sobol [31], who reported the existence of
small epithelial pearls between the layers of the tympanic
membrane.
If the congenital nature of some cholesteatoma is quite
clear, there is much debate about the origin of acquired cholesteatomas. Conceptually, they have been dichotomized into
two groups: primary and secondary. The former would result
from progressive tympanic retraction, which, at some point,
loses its self-cleaning properties and starts to accumulate
desquamated epithelium and keratin. On other hand, secondary cholesteatomas would arise from the migration (invasion) of the external auditory canal epithelium into the
middle ear through a marginal perforation of the tympanic
membrane. Once inside the middle ear, a standard biological
behavior would follow: it would encyst and start producing
keratin [32]. Today, our understanding that the division of
cholesteatomas into these two models, although being quite
didactic and easy to understand, is vastly operationally
incomplete. We will expand on this discussion in specic
sections of this chapter.
Meyerhoff and Truelson [33] tried to classify cholesteatomas according to their pathophysiology, location, ossicular
defects, and presence of complications, also dividing them
into congenital and acquired, the latter being primary, secondary, or tertiary.
Tos [34] proposed an otoscopic classication, dividing
cholesteatomas into:
1—Attic.
2—Pars Tensa I (marginal disease).
3—Pars Tensa II (central disease).
In 1993, the same author proposed another classication,
based on the site of origin of the cholesteatoma, which he
considers an important factor for the surgical procedure and
for the prognosis. This taxonomy presents three categories:
1—Attic cholesteatoma—a retraction of the pars accida
or Shrapnell’s membrane, extending from the attic,
passing through the aditus, and eventually reaching
the antrum, mastoid, or tympanic cavity.
2—Cholesteatoma of the sinus tympani—posterosupe-
rior retraction or perforation of the pars tensa, extend-
ing to the tympanic sinus and posterior recesses.
3—Cholesteatoma of the pars tensa—retraction and
total adhesion of the pars tensa of the tympanic mem-
brane (TM) involving the tympanic orice of the
eustachian tube (ET).
Saleh and Mills [35] proposed another classication,
according to the sites affected by cholesteatoma, characterized as follows:
S1—If the cholesteatoma is restricted to the place where
it started.
S2—when the disease extends to another location.
S3—if it affects three locations.
S4—if installed in four locations.
S5—for cases in which the rst affected site and, in addi-
tion to this, four or more are involved.
Table 42.1 Classication of temporal bone cholesteatomas
Congenital Acquired From the external auditory canal
Eardrum-mastoid Primary
Petrous pyramid Secondary
Cerebellar point angle
Jugular foramen
Intratympanic
These same authors distinguish seven locations used
for this classication: attic and antrum, middle ear, mastoid, ET, labyrinth, and middle fossa.
Saleh and Mills [35] also present a classication of the
condition of the ossicular chain, based on the descriptions of
Wullstein [36] and [37], through the following score:
410
https://t.me/medicina_free
S. S. da Costa et al.
0—intact ossicular chain.
1—Incus is eroded and with discontinuity of the ossicular
chain.
2—Incus and stapes superstructure are eroded.
3—Malleus head and incus are absent, and the stapes
superstructure is eroded.
As for preoperative complications, Saleh and Mills [35]
classied cholesteatoma as:
C0—when there are no complications.
C1—for the occurrence of one complication.
C2—for the existence of two or more.
As complications, the authors consider lateral semicircular canal (LSC) stula, facial paralysis, total sensorineural
hearing loss (SHL), sinus thrombosis, and intracranial
invasion.
Finally, in 2017, a task force of international researchers
was assembled with the aim of standardizing these classications and proposing pathogenesis models. The conclusions
were published in the Journal of International Advances in
Otolaryngology—EAONO/JOS Joint Consensus Statements
on the Denitions, Classication and Staging of Middle Ear
Cholesteatoma [38]. The clinical classication suggested in
the nal consensus of this group contemplates the division of
congenital and acquired cholesteatomas and raties pathogenesis models (Fig.42.3).
Among the conclusions of this consensus and in order to
simplify the extent of cholesteatoma, they propose the socalled STAM system dividing the middle ear and mastoid
space into four sites: difcult access sites (S), tympanic cavity (T), attic (A), and mastoid (M). The difcult access sites
(S) include S1, the supratubal recess (also called the anterior
epitympanum or protympanum), and S2, the sinus tympani.
The posterior border of the attic is the posterior end of the
incus short process or the fossa incudis. The mastoid includes
the antrum and mastoid cells (Fig.42.4).
The EAONO/JOS also proposed a very encompassing
staging system that applies to four types of middle ear cholesteatoma (pars accida cholesteatoma, pars tensa choles-
A
M
S1
S2
T
Fig. 42.4 Divisions of the middle ear space using the STAM system
Fig. 42.3 Schematic drawing
of the clinical classication of
middle ear cholesteatoma
CONGENITAL
Pars tensa cholesteatoma
Pars flaccida
cholesteatoma
CHOLESTEATOMA
ACQUIRED
RETRACTION POCKET
CHOLESTEATOMA
Secondary to TM
Combination of pars flaccida
and pars tensa cholesteatoma
Post surgery
Not mutually exclusive
Recurrent cholesteatoma Residual cholesteatoma
NON-RETRACTION POCKET
CHOLESTEATOMA
Perforation
Following trauma or
iatrogenic causes
UNCLASSIFIABLE
42 Temporal Bone Cholesteatoma: TheFull Picture
https://t.me/medicina_free
411
Table 42.2 The EAONO/JOS staging system applies to four types of
middle ear cholesteatoma
Stage I: Cholesteatoma located in the primary site*
*The site of cholesteatoma origin, i.e., the attic (A) for pars
accida
Cholesteatoma; the tympanic cavity (T) for pars tensa
cholesteatoma, congenital cholesteatoma, and cholesteatoma
secondary to a tena perforation
Stage II: Cholesteatoma involving two or more sites
Stage III: Cholesteatoma with extracranial complications or
pathologic conditions including
Facial palsy,
Labyrinthine stula: with conditions at risk of membranous
labyrinth,
Labyrinthitis,
Postauricular abscess or stula,
zygomatic abscess,
Neck abscess,
Canal wall destruction: more than half the length of the bony ear
canal,
Destruction of the tegmen: with a defect that requires surgical
repair, and
Adhesive otitis: total adhesion of the pars tensa.
Stage IV: Cholesteatoma with intracranial complications including
Purulent meningitis,
Epidural abscess,
Subdural abscess,
Brain abscess,
Sinus thrombosis, and
Brain herniation into the mastoid cavity
The staging system does not apply to petrous bone cholesteatoma
teatoma, congenital cholesteatoma, and cholesteatoma
secondary to a tensa perforation). This system is summarized
in Table42.2 [38].
Histopathology
Cholesteatoma, macroscopically, is a round or oval cystic
lesion with variable conguration and size. Ferlito etal. [4]
characterized cholesteatoma as an epidermoid cyst, with
independent and progressive growth, with destruction of the
adjacent tissues, especially bone, with a tendency to recur.
The advent of transmission electron microscopy made
possible many advances in the knowledge of cellular structure. Using this instrument, in 1972, Lim and Saunders [39]
presented a detailed histological description of cholesteatomas. They described that cholesteatoma has a keratinized
stratied squamous epithelium, with four layers identical to
those of normal epidermis (basal, spinous, granulosa, and
cornea), Langerhans cells (in greater numbers than in normal
epidermis), and keratohyaline granules. They called this epithelium the matrix of the cholesteatoma. They also observed
the presence of a connective tissue, containing collagen
bers, brocytes, and inammatory cells, which was called
perimatrix, which was in contact, in most cases, with a layer
of scaly or cylindrical ciliated cells, remnants of the original
mucosa of the middle ear. In some cases, although the perimatrix was absent at optical microscopy, it was present when
studied with the transmission electronic microscope, showing itself to be extremely thin, with practically absent collagen bers and containing crystals of calcium carbonate. The
cystic content was formed by accumulated keratin, epithelial
debris, and inammatory compounds.
This tripartite structure, matrix, perimatrix, and cystic
content, is well illustrated in Fig.42.5 and will be detailed
below:
Perimatrix
Paludetti etal. [40] described the perimatrix as a mass of
granulation tissue or inamed subepithelial connective tissue. According to Milewski etal. [41], the growth of a cholesteatoma would require angiogenesis in the connective
tissue of the perimatrix, and that cells and substances of the
healing cascade could have an important role in the development and growth of cholesteatomas. These processes would
involve the broblastic growth factor b (b-FGF), which,
according to these authors, could stimulate the production of
collagenase. They also suggested that the persistence of
inammation would cause a permanent healing process in
the perimatrix, the proliferation of broblasts (granulation
tissue), and epithelium (matrix).
Ferlito et al. [4] describe the perimatrix as the most
peripheral portion of the cholesteatoma, consisting of granulation tissue or inammatory subepithelial connective tissue,
with lymphocytes, histiocytes, and neutrophils. Sprekelsen
etal. [42] state that the matrix and perimatrix, in normal or
pathological tissues, are formed by type IV collagen, tenascin, bronectin, b-FGF, and metalloproteinases (MMP).
According to Jacob etal. [43], the increase in the proliferation of the cholesteatoma matrix would be the result of the
inammation process, suggesting that the perimatrix would
be the main factor in the development of cholesteatomas.
Hamzei etal. [44] analyzed 21 cholesteatomas, through
polymerase chain reaction (PCR), immunohistochemistry,
and histology, with the aim of investigating the factors of
stimulation and differentiation of osteoclasts present in cholesteatomas, using the skin of the external acoustic meatus as
a control. Immunohistochemical analysis demonstrated an
increase in osteoclast precursor cells and macrophages in
cholesteatomas. The perimatrix analysis demonstrated that,
in this region of the cholesteatoma, there are all the necessary factors for osteoclastogenesis and for the stimulation of
bone reabsorption.
Briey, we like to dene the perimatrix as a rich inammatory network that surrounds the cholesteatoma. It represents an authentic “battleeld” which may play interesting
Соседние файлы в папке Библиотека им академика М.И. Перельмана