Versão Inglês

Ano:  1966  Vol. 34   Ed. 2  - Abril - Junho - (5º)

Seção: -

Páginas: 59 a 69

THE PATHOLOGY & TREATMENT OF CHOLESTEATOMA AURIS (*)

Autor(es): Sir Terence Cawthorne, M. D., L.L.D., F. R., G. S.

Resumo:
Faz o autor a revisão bibliográfica do assunto que propõe estudar desde DU VERNEY, VIRCHOW, BEZOLD, POLITZER e outros. Cita os autores mais recentes e as teorias sôbre a etiopatogenia do colesteatoma, discutindo estas teorias e dando a sua opinião pessoal quanto ao valor das mesmas. A seguir faz uma tentativa de classificação dos colesteatomas e o seu quadro clínica. Temos assim: 1.º grupo - colesteatoma petrosa: localização profunda no osso temporal, usualmente não infectado, paralisia facial de evolução lenta, hipoacusia, teste calórico negativo, alterações radiológicas. 2.º grupo - colesteatoma atical: perfuração do mico, geralmente infectado e com boa audição, às vêzes bilateral, quase sempre sem alterações radiológicas. 3.º grupo - colesteatoma de mastoide: comumente em crianças; às vêzes com hipotonia muscular e bilateral, com alterações radiológicas. São apresentados três casos clínicos exemplificando os tipos conforme a sua classificação. A técnica cirúrgica é descrita como sendo a aticotomia e ossiculotomia permeatal e que corresponde à técnica operatória descrita por TUMARKIN em 1947. Faz alguns comentários sôbre pormenores de técnica, em relação aos tipos clínicos descritos e também pana a otite média crônica e simples. Considerando os dados bibliográficos, os achados da semiologia clínica e os resultados obtidos pela mesma técnica cirúrgica empregada, o autor sugere a diferença de etiologia, sendo uma de natureza embriogêniea e outra de caráter infeccioso. Salienta que é êste o principal motivo da presente comunicação. A seguir agradece seus vários amigos e colaboradores e apresenta a relação bibliográfica consultada.

Localised collections of cholesteatomatus material in and around the base of the skull were first recognised by Du Verney in 1683, when he referred to them as "steatomes". Cruveilhier in 1829 named them "pearly tumours" and in 1838 Muller was the first to describe one which lay within the petrous bone. In 1855 Virchow used the term "primitive cholesteatoma" and regarded these collections as arising from the ectoderm of the first branchial cleft and he felt that any suppuration within the temporal bone associated with such growths was often the result but never the cause of the cholesteatoma. Habermann in 1888 and Bezold in 1889 impressed no doubt by the number of infected cholesteatoma with attic perforations seen in otological clinic concluded that the collection of cholesteatoma in the attic was the result of an inward growth of the epidermis of the external ear through a perforation of the ear drum into the tympanic cavity, a view which has been accepted by many otologists and pathologists since that day. POLITZER (1926) in Ballin's latest translation of his textbook of the Diseases of the Ear gives an account of cholesteatoma which is both interesting and revealing. It shows how on the one hand POLITZER was clearly attracted to the infective and invasive origin but he could not nor, indeed, did he try to deny the occurrence of an occasional primary cholesteatoma deep in the petrous portion of the temporal bone that could not possibly have grown in through normal tympanic membrane across an unsullied middle ear cleft and through into the petrous bone.

To quote him "Primary cholesteatoma in the temporal bone is on the whole, very rar". I agree with Politzer's statement in so much as an infected attic cholesteatoma is much more frequently encountered by the otologist than an uninfected cholesteatoma deep in the petrous bone. I am however quite unconvinced that cases of uninfected petrous cholesteatoma can be ignored on grounds of rarity when considering the pathology of cholesteatoma auris. That Politzer was strongly attracted to the invasive theory can ben seen from the following quotation from his book:

"The path of invasion of cholesteatoma is not always through the perforated membrane. In a case of cholesteatoma in the mastoid, I could demonstrate the entrance of the epidermis into the spacious cells through a fistula in the cortical layer of the mastoid and through a second fistula in the posterior wall of the meatus".

I have seen two such cases that I find it impossible to accept that in either case the extensive collection of cholesteatoma accompanied by destruction not only of the temporal bone but also of part of the adjacent occipital bone could have been the result of invasion of epidermis from a fistula in the mastoid or in the posterior wall of the external meatus. I have always felt that what I saw was the cholesteatoma eating ias way out of the temporal bone rather than epidermis creeping in.

Like most Otologists I regard the great Adam Politzer as the father of modern otology and I look upon his views with great respect, but like so many sons often do, I beg to be allowed to disagree with him on this point.

Since Politzer's day many leading Otologists and Pathologists, including NAGER ( 1925), RUEDI (1958, 1963), LINDSAY (1959), FRIEDMAN (1959), TUMARKIN (1958), McGUCKIN (1962) and VERH'OVEN (1961) to mention some have favoured the invasive origin of cholesteatoma. Novertheless M- DIAMANT (1953) believes in the neoplastic or embryonic origin of cholesteatoma_ as do McKINZIE (1931), TEED (1936) ond PIRER (1926). HOUSE (1958) and SHEEHY (1959) have reported cases of primary cholesteatoma and every year similar cases of primary uninfected petrous cholesteatomata are reported. So they cannot be so rare as to be ignored when considering the pathology of cholesteatoma auris.

I do not deny that squamous epithelium can invade the tympanum through a perforation in the ear drum, because I have seen this happen but when it does so there is usually a large perforation and only a very thin, whitish membrane covering the inner tympanic wall continuous with the meatal epithelium and I think that it is highly unlikely that this ever develops into a mass of cholesteatoma.

The neuro-surgeon HARVEY CUSHING (1922) suggested that cholesteatoma auris is an epidermoid tumour which may encourage secondary infection and he wrote:

"It is my impression that many of the cholesteatomata reported by Otologists are true epidermoid tumours of the sort under discussion, which originate from epidermal rests laid down in the temporal bone during the early formation of the sense organ which it contains... It is not improbable that in many of the recorded cases the cholesteatoma itself was responsible for the otitis media, rather than the reverse".

I think that HARVEY CUSHING was perfectly right. CRITSCHLEY and FERGUSON in 1928 writing about cerebrospinal epidermoids preferred the use of the term "epidermoid" to cholesteatoma, and though I am bound to agree with this, custom and habit cannot easily be denied and in the hospitals in which I work, the term "primary cholesteatoma" is understood by all who are interested in the condition, In 1931 before the Section of Otology of the Royal Society of Medicine, DAN McKINZIE spoke in favour of an embryonic origin of cholesteatoma and I agree with what he said.

JEFFERSON and SMALLEY in 1938 recorded six intratemporal epidermoids in association with facial palsy, and I discussed these cases with both JEFFERSON and SMALLEY. These were all situated deep in the petrous bone and were uninfected. JEFFERSON deliberately chose to publish the article in the Journal of Laryngology & Otology, in the hope of arousing the interest of otologists in this condition which he regarded as purely otological and not, as was often thought, a condition needing the services of a neuro-surgeon. Many such cases, he felt were mistakenly regarded as acoustic neuromata.

Curiosly enough, I saw one of the patients in JEFFERSON and SMALLEY'S series in the Autumn of 1964. She had been referred to JEFFERSON by Sir CHARLES BALLANCE, as the presenting symptom was, as it nearly always is in a case of petrous cholesteatoma, facial palsy of gradual onset.

In 1961 CAWTHORNE and GRIFFTH reported in Chicago 9 cases of petrous cholesteatoma of which 6 were uninfected.

In 1963, again in Chicago, CAWTHORNE in a paper on "Congenital Cholesteatoma" reported four additional uninfected cases in 3 of which the collection of cholesteatoma was deep in the petrous bone. In one case it was found in the attic, though the tympanic membrane was in "brilliant mint condition" . In two further patients, both children, the cholesteatoma was found mainly in the mastoid antrum and in the few cells leading off it, and since then two further patients with what may be termed 'mastoid cholesteatoma' have been encountered, one on which was reported to me by FOXWEII (1965). Of course, in addition, many patients with an infected attic cholesteatoma have been seen and will be considered further in the surgical treatment of the condition. Thus, I recognise collections of cholesteatoma which are sometimes located deep in the petrous bone, often more superficially in the epitympanum or attic or occasionally in the mastoid antrum and adjoining cells. This third group may be a sub-division of the second or evem the first but all I believe originate in the same way namely as an embryonic remnant. Finally, any of these collections may be complicated by infection but are, I firmly believe, never the result of infection. The clinical features of these three groups can be summerised as follows:

1. Petrous Cholesteatoma
Deep in petrous bone
Usually uninfected
Facial palsy of slow onset Deafness
Caloric absent X-ray changes

2. Attic Cholesteatorna
Attic perforation
Usually infected
Usually good hearing
Sometimes bilateral
Often no X-ray changes

3. Mastoid Cholesteatorna
Usually children
Sometimes facial weakness
Sometimes bilateral
X-ray changes.

The following cases histories give examples of primary petrous cholesteatoma.

CASE I.

A schoolboy, aged II years. At the age of 10 years an insidious and slowly progressive right facial weakness was noticed. A provisional diagnosis of Bell's palsy was made and electrical treatment was started, but it was without benefit. After a few months the child was referred for further investigation.

There was no history of former ear disease. The right tympanic membrane showed a postero-inferior bulge with the appearance of a solid whitish mass pushing against, but not through, the tympanic membrane. There was a profound right perceptivo deafness and no response to caloric stimutation of the right car. There was a weakness of the whole musculature of the right side of the face Bell's phenomenon was present, and the taste over the anterior two-thirds of the right side of the tongue was impaired. No other abnormality was found. An X-ray of the skull appeared normal, but coned views of the petrous temporal bones demonstrated some sclerosis, with arcas of breakdown of periantral cells. This process appeared to extend upward, and the Stenvers projection revealed a break in continuity otf the outline of the bone, suggesting the possibility of intracranial involvement.

The right petrous bone was explored through a postauricular incision. A whitish uninfected mass of cholesteatoma was removed from the vestibular part of the bony labyrinth. The dura of the middle fossa was exposed. The cholesteatoma had spread into the tympanic cavity; it was removed, and the facial nerve was revealed lying in the bottom of the cavity; it was swollen but intact. The incision was closed. There was no recovery of facial nerve function. Subseduenty the cavity-was reopened and the sheath of the nerve slit open; eventually some recovery of facial movement took placo.

COMMENT

This primary epidermoid arose around the posterior part of the labyrinth and spread forwad into the inner ear, upward into the middle fossa, and lateraly finto the tympanic cavity, but the tympanic membrane was not perforated.

CASE 4.

A housewife, aged 55. For 3 years this patient was aware of a twithing of the muscles around her eye and a progressive left facial weakness. She gane a history of long-standing deafness, which she attributed to a brief attack of car infection earlier in life. From time to time she had become giddy cm looking up or on sudden movements of the head, and had noticed a pain behind the left ear.

On examination, the left tympanic membrane showed some scarring, which was in keeping with the history of an carlier attack of otitis media. Tests of cochlear function revealed on the left side a profound perceptive deafness. There was no reaction in the left ear to caloric stimulation. The left facial nerve palsy was complete. The right ear was normal.

X-rays of the skull and petrous bones were difficult to interpret, but tomographs showed a clear translucent defect lying medial to the semi-circular canal and extending into the lateral portion of the internal auditory meatus. The appearances were those of a primary epidermoid of the right petrous bone.

The petrous bone was explored through an endaural incision. Although the tympanic membrane showed some scarring, there was no cholesteatoma within the middle ear. But deeper, and lying quite separate from the tympanic cavity, an extensive epidermoid was encountered. This was removed with several sequestrated portions of the petrous bone. The dura of the middle fossa was low-lying and exposed. The facial nerve was seen lying within the cavity. The wound was closed without drainage.

COMMENT.

This primary epidermoid arose in a patient who had an attack of otitis media earlier in life. The deep situation, its total freedom from infection, and the demonstrated absence of continuity between the cholesteatoma cavity and the middle ear, permit the inclusion of this case in this series despite the brief history of otitis media.

CASE 3.

Draper, aged 42. This patient complained of a slight left-sided facial weakness for 2 months and some watering of the left eye. For 14 years she had been totally deaf in the left ear and had observed loss of steadiness on sudden movements of the head. She gave a history of an episode of otorrhea in the past. On examination, the left ear showed scarring and in-drawing of the tympanic membrane. Cochlear function was absent in the left ear. A caloric test was not performed on account of the condition of the tympanic membrane.

X-rays revealed a spherical translucency 5 cm in diameter in the posterior part of the left petrous temporal bone. It lay medial to the mastoid antrum, and from these appearances a diagnosis of a primary epidermoid was made.

At operation a large uninfected mass of white pearly cholesteatoma was found lying medial to the labyrinth, spreading anteriorly into Trautman's triangle. It was removed. The facial nerve was not seen. The wound was closed without drainage. Subsequently there was some recovery of facial movements.

COMMENT.

It is considered that this was a primary epidermoid. This cannot be claimed as a proved case on account of the history of otitis media and the proximity of the cholesteatoma to the mastoid antrum. Any connection with the tympanic cavity suas excluded at the time of operation.

Once cholesteatoma auris is suspected, it must be explored, because, as is well known, cholesteatoma has the power of gnawing its way through the temporal bone, increasing in size as it gnaws and if it becomes infected may lead to intracranial complications. The reason that the infected attic cholesteatoma is no much in the mind of the otogist is because it is comparatively common and the primary growth in the attic does not have far to grow to reach the outer ear when it is liable to become infected and will present as anotological disorder.

MANAGEMENT:

If the cholesteatoma is petrous and uninfected then, through a superficial endaural incision, the petrous bone is explored, the cholesteatoma is removed, and the wound of entry is then closed without drainage. If the cholesteatoma is in the attic it is more often than not infected and some form of drainage is needed. The traditional way of dealing with such a case is by means of a radical mastoid.

However, I have found that most infected attic cholesteatomata respond quickly and well to removal of the outer attic wall, enlarging of the aditus, removal of the incus and all the malleus together with the cholesteatoma. The remains of the tympanic membrane is allowed to cover the head of the stapes, which is left undisturbed.

A. Permeatal Atticotomy and Ossiculectomy. Steps:

1. Stapedectomy approach.
2. Outer attic wall removed.
3. Aditus enlarged.
4. Incus and malleus (including handle) removed.
5. Cholesteatoma removed.
6. Flap cut off.
7. Remains of tympanic membrane placed of stapes.

This operation is based on TUMARKIN'S transmeatal atticotomy (1947). The approach is as for the stapedectomy operation via an aural speculum, but the flap of deep meatal skin is discarded, as so often it is infected, and to leave it behind will hinder drainage and encourage infection. The incus and the whole of the malleus are removed. The stapes is left undisturbed. Any cholesteatoma and lining membrane is carefully hooked out with a double-angled needle (Cawthorne No. 4 needle).

The cavity is syringed out, Sofradex solution is instilled and a piece of wool is placed in the outer meatus.

Sofradex ear drops are instilled twice a day for 4 days by which time four-fifths of the ears are quite dry and remam dry, so long as water is prevented from entering the ear. Half of the remaining fifth will subseduently need a more extensive operation to eradicate the disease, so all should be warned that what I refer to as "permeatal atticotomy" and ossiculectomy may possibly be a preliminary and limited operation which, however, in four-fifths of the cases is the only operation neaded to obtain a dry and safe ear.

It calls for a stay in hospital of there days and is so regularly successful as regards the eradication of the disease and obtaining a procedure of choice.

B. Permeatal Atticotomy and Ossiculectomy for Attic Cholesteatoma.

Dry - 24
Moist - 5+
+3 subsequently needed a radical mastoid.

If, as sometimes happens, both ears are affected and particularly if the ear to be operated on is the better-hearing ear, then permeatal atticotomy without ossiculectomy will have to be tried and may result in a dry ear with no further impairment of hearing.

It may be possible to deal with the mastoid cholesteatoma by the permeatal route, especially when the aditus can be enlarged. If X-rays reveal a large cavity in the mastoid, then the wider exposure afforded by an endaural incision will be needed.

Although this paper is about cholesteatoma. I must refer to the use of permeatal atticotomy and ossiculectomy for chronic otitis media without cholesteatoma. In such cases the diseases is more tubo-tympanic and there may be granulations and even polypi in the middle ear, and I have found that the chance of achieving a dry ear in such cases is little more than 50%.

C. Permeatal Atticotomy and Ossiculectomy for Chronic Ear Infection.
Dry - 5
Moist - 41
+1 subsequently needed radical.

This striking difference in the healing rate between cholesteatoma and infection after a limited operation suggests, as I do, that the two disorders have a different etiology; the one being embrvonic and the other infective. This then is the main theme of my communication.

I am grateful to all those from whom I have learnt about this interesting though controversial condition and I hope that out of this contribution will throw a little more light upon a subject which has so often been obscured by heat and sound.

I am particularly grateful to Dr. Hugh-Davies and Dr. James Bull of the Lysholm Radiological Department of the National Hospital Queen Square, who have shared our enthusiasm in this group of cases and have provided us with so many beautiful pictures of the petrous bone, and to Mr. Briam Pickard who has helped in this work.

I am also sensible of the fact that working at a Neurological Hospital has, thanks to my colleagues who have so generously and freely referred their cases to me, given me a sufficient variety of cases of cholesteatoma auris to convice me that they have a common etiology and the they need simple exposure and excision with, as a rule, as little removal of bones as is compatible with eradication of the disease.

Finally I would like to pay tribute to the memory of Adrian Griffith whose enthusiasm, energy and ability stimulated me to embark on this fascinating subject.

REFERENCES

BEZOLD, F. (1889) - Cholesteatom, Perforation der Membrana Flaccida Sharapnelli und Tuberverschltiss, eine atiologieche studie. - Zeitschrift f. Chrenheilk. 20, 5-28.
BIBER, J. J. (1962) - Keratosis obturans versus the so-called cholesteatoma of the external meatus. - 3rd Congrèss Greco-Yougoslav. O.N.O. p. 13-19.
CAWTORNE, T. & GRIFFITH, A. (1961) - Primary Cholesteatoma of the temporal bone. Arch. Otolaryng. 78, 252.
CAWTHORNE, T. (1963) - Congenital cholesteatoma. Arch. Otolaryng. 78, 248.
CRITCHLEY, M. & FERGUSON, F. (1928) - The Cerebro-Spinal Epidermoids (Cholesteatoma), Brain, 51, 334.
CRUVEILHIER, J. (1829) - Anatomie pathologique du corpe humain, Paris, J. 13. Bailliere, Vol. 1, Bk. 2. 341.
CUSHING, H. (1922) - A Large Epidermal Cholesteatoma of the parieto-occipital region deforming the left hemisphere without cerebral symptons. Surg. Gynec. & Obstet. 34, 557.
DAY, K. (1955) - A primary cholesteatoma of the middle ear. Aun. Otol. Rhin. & Laryng. 64, 161.
DIAMANT, M. (1953) - Cholesteatoma. An Analysis of Fact and Figment. Acta Genet (Basel). 4, 151.
DU VERNEY, J. G. (1683) - Traite de l'organe de l'ouie, Paris E. Michallet.
FERNANDEZ, C., LINDSAY, J. R., & MOSKOWITZ; Al. (191-1) - Some observations of the pathogenesis of middle-ear cholesteatoma. Arch. Otolaryng. 69, 537.
FOXWELL, P. B. (1965) - Personal communication,
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HABERMANN, J. (1888) - Zur Entescliung des Cholestealomas der Mittelhors. Arch. Ohr. Has Kehlkopfheilk 27, 42.
HOUSE, H. P. (1953) - An apparent primary cholesteatoma. Laryngoscope, 63, 721.
JEFFERSON, G. & SMALLEY, A. A. (1938) - Progressivo Facial Palsy produced by intratemporal epidermoids. J. Laryng. 53, 417.
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McGUCKIN, F. (1962)
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POLITZER, A. (1926) - Diseases of the Ear. Balliere, Tindall & Cox London, p. 416.
RUEDI, L. (1958) - Cholesteatosis of the Attic. J. Laryng. 72, 593.
RUEDI, L. (1963) - Acquired cholesteatoma. Arch. Otolaryng, 78, 252.
SHEEHY, J. (1959) - True cholesteatoma. Arch. Otolaryng. 69, 57.
TEED, R. (1936) - Cholesteatoma verum tympani. Arch. Otolaryng, 24, 455.
TUMARKIN, A. (1938) - A contribution to the study of middle-ear suppuration with special reference to the pathogeny and treatment of cholesteatoma. J. Laryng. 53, 756.
TUMARKIN, A. (1948) - Transméatal Atticotomy. J. Laryng. & Oto, 62, 316.
TUMARKIN, A. (1950) - Attic supuration. J. Laryng. 64, 611-624.
TUMARKIN, A. (1958) - Attic Cholesteatosis. J. Laryng. 72, 593.
TYTUS, J. S. & PENNYBACKER (1956) - Pearly tumours in relation to the Central Nervous System. J. Neurol. Neurosurg. Psychiat. 19, 421.
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VIRCHOW, R. (1859) - Veber Perlgeschwulste. Virchow's Arch. f. path. anat. 8, 371.

(*) XIV Congresso Brasileiro de O.R.L. Rio de Janeiro. Outubro, 1965.

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