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Cholesteatoma

Medial

to an Intact

Tympanic

Membrane

in 34 Young

Children

Richard

H. Schwartz,

MD, Kenneth

M. Grundfast,

MD,

Bruce

Feldman,

MD, Richard

E. Linde,

MD, and

Karen

L. Hermansen,

MD

From the Department of Pediatrics and Department of Surgery, Section of Otolaryngology, Fairfax Hospital, Falls Church, Virginia, and Department of Otolaryngology, Children’s Hospital National Medical Center, Washington, DC

ABSTRACT. Thirty-five cholesteatomas medial to intact

eardrums were treated in 34 children between 1976 and 1982. Six (18%) children had never had a documented episode of otitis media. Seventeen (50%) children, in whom the lesion was diagnosed at an early stage, under-went simple excision of the cholesteatoma without the need for extensive middle ear surgery. Findings from postoperative audiograms were normal for all such chil-dren. Cholesteatoma has recurred in eight (23%) children to date. Most recurrences were diagnosed 15 months or

less after surgery. Routine careful otoscopic examination is essential in order to discover cholesteatoma at an early

stage and to avoid hearing loss and the need for extensive

otomastoid surgery. In order to perform an accurate examination of the eardrum, a halogen-illuminated oto-scope and pneumo-otoscopy should be used by the pedia-trician routinely. Particular attention should be paid to

the posterior-superior quadrant of the tympanic

mem-brane where a cholesteatoma is usually located. Pediatrics 1984;74:236-240; cholesteatoma, intact tympanic

mem-brane.

A cholesteatoma (keratoma) is a nonmalignant

accumulation of keratin medial to the outer layer of the tympanic membrane, enclosed, at least orig-inally, by a membranous stroma of connective tis-sue. It may be found in the middle ear cleft, in the mastoid, or within the layers of the tympanic

mem-brane. Gray’ states that it is simply “skin in the

wrong place.” Although a cholesteatoma is histolog-ically benign, it can enlarge and erode the middle ear structures as well as invade the mastoid bone.

Aural cholesteatomas have three distinguishing

fea-tures: (1) they continue to accumulate squamous

Received for publication Oct 18, 1982; accepted April 29, 1983. Reprint requests to (R.H.S.) 410 Maple Aye, West, Vienna, VA 22180.

PEDIATRICS (ISSN 0031 4005). Copyright © 1984 by the American Academy of Pediatrics,

debris within the limiting membrane and as a result they slowly grow larger; (2) they may impinge upon and ultimately destroy vital middle ear structures, and in time, may invade sites adjacent to the middle ear and extend beyond the confines of the temporal bone; and (3) if incompletely extirpated, they tend to enlarge, particularly in children.

In infants, cholesteatomas can arise from “epi-thelial rests” located within the middle ear cleft. Such epithelia! rests have been called mesotym-panic cholesteatomas; they have a high recurrence rate, leading to significant problems in hearing in

many children.

Cholesteatomas also may be loculated within the

layers of the tympanic membrane; when they occur

in this form they have been called

intramembran-ous cholesteatomas. An intramembranous chole-steatoma appears as a small pearl-like cyst trapped within the tympanic membrane. If detected early, an intramembranous cholesteatoma can be excised

easily and does not seem to have as high a recur-rence rate as the more sinister mesotympanic chol-esteatoma. It is important to recognize either an intramembranous or a mesotympanic cholestea-toma before perforation of the eardrum and chronic otorrhea occur in order to prevent extensive

de-struction of middle ear structures.

The medical records of 34 children who

under-went excision of cholesteatomas medial to intact

tympanic membranes were reviewed. Analysis of the data indicates that cholesteatomas were de-tected even in very young children. Cholesteatomas were often found to be associated with persistent

otitis media with effusion. They were at first

(2)

PATIENT POPULATION

We reviewed the medical records of children

treated for cholesteatoma at a large community hospital and at a major urban children’s hospital. Thirty-four children underwent excision of chole-steatoma medial to intact tympanic membranes between 1976 and 1982; one child had bilateral cholesteatomas. The age range of the 34 children

was as follows: five (15%) were less than 2 years old; 23 (67%) were 2 to 8 years old; and six (18%) were more than 8 years old. Mean age was 4.7 years. There were 25 boys and nine girls in the study;

these 34 children represent 18% of 184 patients treated surgically for cholesteatoma between 1976 and 1982.

DIAGNOSIS

In 23/34 (68%) children, cholesteatomas were

diagnosed by an otolaryngologist in the office (Ta-ble 1). In 14 of these children, the lesion was diag-nosed following referral of the child by a pediatri-cian, local health department, or craniofacial

anom-aly clinic. In four children, the lesions were discov-ered by the otolaryngologist during a surgical fol-low-up visit after extrusion of tympanostomy tubes.

In the remaining five (18%) children, the

cholestea-toma was unsuspected by the otolaryngologist

dur-ing the office examination and was discovered at the time of surgery for tympanostomy tubes. Eleven

(32%) cholesteatomas were diagnosed by nine dif-ferent pediatricians; six of these nine pediatricians

use a pneumatic otoscope routinely.

Three of the children in the study had obvious clefts of the soft palate and uvula, but no other

TABLE 1. Diagnosis of Cholesteatoma Medial to Intact

Tympanic Membrane in 34 Children

Diagnosis by No. of Children

Subtotal Total

Otolaryngologist 23 (68%)

1. Routine follow-up otolaryngo- 4

logic visit after extrusion of tympanostomy tube

2. Consultation

a. Pediatrician referral for per- 10 sistent otitis media with ef-fusion

b. Health department referral 3

because child failed school hearing screening examina-tion

c. Orofacial clinic referral for 1 routine otoscopic

examina-tion

3. Surgery to place tympanostomy 5

tubes (previously unsuspected cholesteatoma)

Pediatrician 11 (32%)

craniofacial anomalies were noted. Seventeen of the children had received multiple courses of antibiotic therapy for recurrent episodes of otitis media prior to the time when the diagnosis of cholesteatoma was made; the other 17 children had infrequent episodes of acute otitis media. No prior otolaryn-gologic surgery (tympanostomy tubes,

adenoidec-tomy, myringotomy) was documented for 26 (76%) of the children. Tympanostomy tubes had been inserted in seven children and tympanocentesis without insertion of ventilation tubes had been performed in one additional child. Within 2 months prior to surgical excision of the cholesteatoma, au-diograms were obtained for 27 (80%) of the chil-dren. Preoperative audiogram findings were normal in eight (24%) of the children; 11 (32%) children had a mild hearing loss, and eight (24%) had a moderate hearing loss.

RESULTS

During a 6-year period ending in early 1982, a total of 184 children aged 12 years or younger had

surgery for cholesteatomas of the middle ear and mastoid. There were 100 children treated at Chil-dren’s Hospital National Medical Center and 84 children treated at Fairfax Hospital, a suburban community hospital. Ofthese 184 children, 35 chol-esteatomas medial to intact tympanic membranes

were excised from 34 children (18%).

Cholesteatomas medial to an intact tympanic membrane were entirely intramembranous in seven children, one of whom had bilateral

intramembra-nous cholesteatomas. Cholesteatomas were found more often in the left ear (59%) than in the right ear (41%). Thirty of the lesions were seen behind the superior half of the tympanic membrane; the remainder were seen behind the inferior half. Ex-cision of cholesteatomas between 1976 and 1982

was as follows: Children’s Hospital National

Med-ical Center-1978, one; 1979, one; 1980, seven;

1981, seven (total = 16); Fairfax Hospital-1976,

one; 1977, three; 1978, five; 1979, two; 1980, three;

1981, three; 1982, one (total = 18). Note that only

four cholesteatomas were excised in the first 2 years of the study in contrast to 20 lesions excised during 1980 and 1981.

Persistent otitis media with effusion in addition to cholesteatoma was noted at surgery in 22 (54%) children. No middle ear effusion was found in eight children who had cholesteatomas. No information concerhing middle ear effusion was found in the

charts

of

four patients. Ossicular damage was found

in 16 children; this included erosion of the incus in

12 (35%) children and destruction of the incus in

(3)

No. of Children

Subtotal Total %

15 7

2 6

TABLE 3. Findings on Postoperative Audiograms of 34

Children with Cholesteatoma Medial to Intact Tympanic

Membrane

DISCUSSION

in 17 (50%) children (including the child with

bi-lateral cholesteatomas). Tympanomastoidectomy

was necessary in 14 (41%) of the patients, and a

radical mastoidectomy was performed in three

(9%). The findings at the time of surgery are shown in Table 2.

Findings from postoperative audiograms were normal in 23 children and abnormal in eight chil-dren; a mild hearing loss (20 to 40 dB) was present

in four children (Table 3). All of the patients who

had abnormal findings on postoperative audi-ograms had undergone extensive otomastoid sur-gery. Growth of residual cholesteatoma was noted

in 8/31 (26%) children who had been followed for a minimum of 1 year after surgery. There was no

recurrence in 23 children and insufficient follow-up

in the other three.

TABLE 2. Findings at Time of Surgery for

Cholestea-toma Medial to Intact Tympanic Membrane in 34 Chil-dren

History of persistent otitis media

(OM) with effusion

OM with effusion at time of

sur-gery

History of recurrent acute OM

No history of recurrent acute

OM

No OM with effusion at time of

surgery

History of acute OM*

No history of recurrent acute

OM

Unknown history

Surgical procedure for excision of cholesteatoma

In toto*

Tympanomastoidectomy

Radical mastoidectomy

Findings at time of surgery

Ossicular damage present 16 47

Incus erosion 12

Incus destruction 4

No ossicular damage* 18 53

* Includes child with bilateral intratympanic

cholestea-tomas.

Audiograms No. of Ears %

-Normal 23* 69

Abnormal 23

Mild hearing loss 4

Moderate hearing loss 4

Not noted on chart 3 8

* Child with bilateral cholesteatomas had normal findings

on postoperative audiograms.

Since 1959, at least 22 reports (in English) of

children with cholesteatomas medial to intact tym-panic membranes have appeared in the

otolaryn-gologic literature. With the exception of three

stud-ies,24 the 12 reports prior to 1978 each reported on

fewer than four cases of cholesteatoma medial to

an intact tympanic membrane. Since 1979, how-ever, ten articles describing a total of 67 children with this lesion have been published.5’4 Finnish otolaryngologists6 found 13 such children, and

American otolaryngologists reported 35 additional

cases.11”2 Although information concerning the method of diagnosis is often lacking, at least 11/67 children were referred to an otolaryngologist be-cause they failed a hearing test administered in school, and eight children were referred after pedia-tricians had detected something unusual behind the tympanic membrane.25’8’15 At least 14 children had no prior history of otitis media.2’4”2”618 House and Sheehy” state that complications of cholesteatoma such as a labyrinthine fistula are less common when cholesteatomas medial to intact tympanic

mem-branes are diagnosed at an early stage.

22 65 Sobel et al’2 presented data on five children with intramembranous cholesteatomas and ten children with mesotympanic cholesteatomas medial to in-tact tympanic membranes. The lesions were

diag-8 23 nosed1980. Theseduring authorsa 27-monthstress periodthat suchending cholesteato-in March

mas are more common than is generally recognized, and they recommend that the physician perform careful, frequent follow-up otoscopy for children

4 12 with repeated attacks of otitis media in order to

detect these lesions at an early stage. Our data and our recommendations are in accord with those of

17 50

14 41 Sobel et al.

3 9 More than half (58%) of the cholesteatomas in our study were diagnosed and treated between 1980 and 1982, the last 2 years of our study. It is unlikely that the great number of cholesteatomas medial to intact tympanic membranes that were discovered during the study period could be an iatrogenic com-plication of tympanostomy tube surgery, because 26 (76%) of the patients had not undergone any otolaryngologic surgery prior to excision of the cholesteatoma. As a result of early diagnosis, many cholesteatomas could be removed simply by a tym-panotomy approach, thus precluding great loss of auditory acuity or the need for radical surgery.

(4)

residual cholesteatomas in adults,’3 a “second-look” operation 6 to 12 months following initial excision may be necessary to remove any residual middle ear cholesteatoma.’ Smyth’9 found residual chole-steatomas in 24% of a large series of children with a past history of chronic otitis media who had had

excision of their cholesteatomas. In those children who underwent planned reexploration ofthe middle ear space and epitympanum, 40% had evidence of residual cholesteatoma. Only 21% of the adult pa-tients in Smyth’s series who underwent a planned

reexploration tympanotomy had residual

chole-steatoma.

DIAGNOSIS OF CHOLESTEATOMA IN

PEDIATRICIAN’S OFFICE

The tentative diagnosis of intramembranous or

mesotympanic cholesteatoma is made primarily by pneumo-otoscopy using an airtight halogen-illumi-nated otoscope. Generally, a whitish or golden mass

is seen behind a translucent eardrum. A mass with ill-defined borders often may be seen in the

poste-nor-superior quadrant of the middle ear even when there is concomitant persistent otitis media with effusion. The second type of cholesteatoma, the intramembranous type, appears as a pearl-like cyst

in the tympanic membrane.

The differential diagnosis of mesotympanic chol-esteatoma includes a sclerotic area in the tympanic

membrane as well as a prominent lateral process of

the malleus, a prominent incudostapedial joint, and the posterior-superior retraction pocket. All of these may simulate a mesotympanic cholesteatoma.

A thorough knowledge of the anatomy of a normal

tympanic membrane and its landmarks is necessary to avoid overzealous referral to an otolaryngologist

because of an unusual finding medial to the ear-drum.

The otolaryngologic literature contains several

theories on the pathogenesis of cholesteatoma.5 For

example, some intratympanic cholesteatomas may be associated with a retraction pocket, a well-cir-cumscribed, deeply retracted area of the

posterior-superior quadrant of the eardrum. Skin drawn into the middle ear cleft may be pinched off and begin to proliferate in the mesotympanum. Why

chole-steatomas occur, however, is of less importance to the clinician than the fact that they do occur.

Cholesteatomas medial to an intact membrane in

young children are not rare lesions. For the past 3

years, in the office practice of one author (R.H.S.),

cholesteatoma has been diagnosed at least as

fre-quently as acute bacteria! meningitis. If overlooked during childhood, cholesteatomas may ultimately announce themselves with foul-smelling, chronic otorrhea through a perforated tympanic membrane

as the more familiar secondary acquired cholestea-tomas. Such a late stage of disease may be avoided by earlier diagnosis. We believe that the primary care physician must learn to perform routinely a careful, systematic, otoscopic examination with particular attention to the area around the lateral malleolar process and the posterior-superior quad-rant of the eardrum, an area often overlooked by a

cursory otoscopic examination. Unexplained con-ductive hearing loss after tympanostomy tube

sur-gery also may be caused by an undiagnosed chole-steatoma. When diagnosed at an early stage by an alert pediatrician who takes the time to examine carefully every child’s eardrum, cholesteatomas can be excised, hopefully, before such lesions cause extensive destruction of middle ear structures and

significant hearing loss.

ACKNOWLEDGMENTS

The help of the medical librarians of the Medical Records section at Fairfax Hospital, Falls Church, Vir-ginia is appreciated. This manuscript incorporates sug-gestions made by Timothy Reichert, MD, James Sheehy,

MD, Jacob Sad#{233},MD, and Tauno Palva, MD. Diana B.

Mathis and Linda Hartekar provided editorial support.

REFERENCES

1. Gray JD: The treatment of cholesteatoma in children. Proc Roy Soc Med 1964;57:769-771

2. Derlacki EL, Clemis JD: Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965;74:706-727

3, Derlacki EL, Harrison WH, Clemis JD: Congenital chole-steatoma of the middle ear and mastoid: A second report presenting seven additional cases. Laryngoscope 1968; 78:1050-1078

4. Derlacki EL: Congenital cholesteatoma of the middle ear and mastoid: A third report. Arch Otokzryngol 1973;97:177-182

5, Smith R, Moran WB: Tympanic membrane keratoma (cholesteatoma) in children with no prior otologic surgery. Laryngoscope 1977;87:237-245

6. Palva A, Karma P, Karja J: Cholesteatoma in children. Arch Otolaryngol 1977;103:74-77

7, Wright WK: Management of otitic cholesteatomas. Arch

Otolaryngol 1977;103:144-147

8. Wingert RH, McCurdy JA, Hays LL: Cholesteatoma: Con-genital and acquired. Milit Med 1978;143:714-719

9, Curtis AW: Congenital middle ear cholesteatomas: Two unusual cases and a review of the literature. Laryngoscope 1979;89:1 159-1165

10. Ruedi L: Pathogenesis and surgical treatment of the middle ear cholesteatoma. Acta Otolaryngol Suppi 1979;361:1-45 11. House JW, Sheehy JL: Cholesteatoma with intact tympanic

membrane: A report of 41 cases. Laryngoscope 1980;90:70-76

12. Sobol SM, Reichert TJ, Faw KD, et al: Intramembranous and mesotympanic cholesteatomas associated with an intact tympanic membrane in children. Ann Otol Rhinol Laryngol 1980;89:312-317

13. Glasscock ME, Dickens JRE, Wiet R: Cholesteatoma in children. Laryngoscope 1980;91:1743-1753

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Laryngoscope 1981;91:1145-1149 intact tympanic membranes: Clinical studies. Acta Otokir-15. Brownlie Smith A: Congenital cholesteatoma. J Laryngol yngol 1970; (suppl 264) p 25

Otol 1968;82:1049-1051 18. Jahrsdoerfer RA, Fitz-Hugh GS: Congenital middle ear

dis-16. Laff HI: Two cases of primary latent cholesteatoma diag- ease. Va Med Mon 1971;98:592-601

nosed and treated by tympanoplasty: Discussion of patho- 19. Smyth GDL: Postoperative cholesteatoma. in McCabe BF, genesis and management of cholesteatoma. Trans Am Lar- Abramson M, Sade J (eds): First International Conference yngol Rhinol Otol Soc 1959;69:369-380 on Cholesteatorna. Birmingham, AL, Aesculapius Publishing 17. Elbrond 0: Defects of the auditory ossicles in ears with Co, 1977, pp 355-362

GEORGE

CATLIN

HAS

ADVICE

FOR

YOUNG

PEOPLE

George Catlin, that prodigious 19th century traveler and chronicler of North American Indian tribes by pen and artist’s brush, was deeply impressed by the exuberant good health of his subjects, and he was morbidly depressed by the devastating effect of the coming of the degenerate white man with his deadly

gifts to the unsuspecting native populations.

He was convinced he had found an obvious source for the overwhelming

differences in the physical well-being of the two races and published, late in life, when he was 65 years old, his almost hysterical observations in a slender treatise: The Breath of Life, with 25 illustrations, published by John Wiley, New

York, 1861, price 25& This was followed in 1870 by another in the same vein: Close Your Mouth-Save Your Life.

“Breath of Life or mal-respiration and its effects upon the enjoyments and life of Man” No person on earth who reads this little work will condemn it; it is only a question how many millions may look through it and benefit themselves by adopting its precepts.

We learn that in London and other large towns in England and cities of the continent, on an

average, one-half of the human race die before they reach the age of 5 years and one-half of the remainder die before they reach the age of 25.

There is certainly no parallel to it to be found amongst North American Tribes where they are living according to their primitive modes.

The contrast between the two races, whose terms of natural life are intended to be equal, shows

that in civilized races there must be some hidden cause of disease not yet fully appreciated.

This cause I believe to be the simple neglect to secure the vital and intended advantages to be derived from quiet and natural sleep, the great physician and restorer of mankind both savage and civil, as well as of brute creations.

There is no animal in nature except man that sleeps with the mouth open, and with mankind, I believe the habit, which is not natural, is generally confined to civilized communities where the habit is easily contracted. Man was made, like other animals, to sleep with his mouth shut and

strong corroboration of this fact is met with amongst the North American Indians who strictly adhere to nature’s law in this respect and show the beneficial results in their fine and manly forms

and exemption from mental and physical diseases.

The savage infant breathing the natural and wholesome air, closes its mouth during sleep. But

when we turn to civilized life, our pity is enlisted for the tender germs of humanity brought forth

and caressed in smothered atmospheres which they can only breathe with their mouths wide open,

a habit which is to shorten their days with the croup of infancy, or turn their brains to idiocy or

lunacy and their spines to curvatures. Children had better sleep with their heads out of the window than in their mother’s arms.

The poor Madonna of the Plains who watches her infant and presses its lips together as it sleeps in its cradle attracts the ridicule or pity of the passer-by, but secures the habit in her progeny which

enables them to command the admiration and envy of the world.

Boys and Girls, I advise you to bear in mind the awful bills of mortality amongst civilized societies and realize the dangerous race which civilized man runs in life-how very few live to the

age designed by Nature, how many perish in infancy long before they are of your age-and contrast all of these to those in the Wild Indians, who, by nature, are no stronger than we but live to good

old age with comparatively few bodily pains in life and their teeth almost uniformly regular and sound without the aid of dentists and tooth brushes.

. . . and if I were to endeavor to bequeathe to posterity the most important motto which human language can convey, it should be in three words: SHUT YOUR MOUTH.

Iwould engrave it in every nursery and on every bedpost in the universe where its meaning could not be mistaken and if obeyed, its importance would soon be realized.

(6)

1984;74;236

Pediatrics

L. Hermansen

Richard H. Schwartz, Kenneth M. Grundfast, Bruce Feldman, Richard E. Linde and Karen

Cholesteatoma Medial to an Intact Tympanic Membrane in 34 Young Children

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1984;74;236

Pediatrics

L. Hermansen

Richard H. Schwartz, Kenneth M. Grundfast, Bruce Feldman, Richard E. Linde and Karen

Cholesteatoma Medial to an Intact Tympanic Membrane in 34 Young Children

http://pediatrics.aappublications.org/content/74/2/236

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