International Journal of Pediatric Otorhinolaryngology, 22 (1991) 213-218 0 1991 Ekevier Science Publishers B.V. All rights reserved 0165876/91/$03.50

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PEDOT 00740

Research Reports

Late results after cholesteatoma surgery in early childhood

Peter Rignk, Ulf Renvall and Anders Tjellstrijm

Department of Otolaryngology, Sahlgren S Hospital, University of Gothenburg, Gothenburg (Sweden)

(Received 21 June 1990) (Revised version received 5 March 1991)

(Accepted 11 March 1991)

Key words: Cholesteatoma surgery; incidence

Abstract

The incidence of cholesteatoma in children living in an urban area of western Sweden has been studied. The total population of this area is 470,000 out of which 100,000 are children, 16 years or younger. The charts of all the children undergo- ing cholesteatoma surgery during the time period from 1977 to 1986 were collected and analyzed. Only those patients who had not been exposed to cholesteatoma surgery before were included. In a follow-up study the clinical situation and hearing level have been evaluated 4 years or more after surgery. Nineteen patients, that is an incidence of 0.4 children out of 100,000 inhabitants each year, were found meeting these criteria with an irregular distribution over the 10 year period. During the first two years 11 cases were found, but in the following 8 years only 8 new cases were identified. Intact wall tympanoplasty was used in 10 cases and canal wall down operation in 9 cases. Residual cholesteatoma was detected in 6 cases and recurrent cholesteatoma in one case. Six of these had an intact wall tympanoplasty and all but one were later reoperated with a canal wall down approach. The study reveals a decrease in the incidence of cholesteatoma in children over a lo-year period. The more frequent use of tympanometry and otomicroscopy is a possible explanation.

Introduction

There is a general agreement that cholesteatoma in children is more aggressive than in adults with a higher recurrence rate and more extensive and rapid growth

Correspondence: P. Rigrkr, Department of Otorhinolaryngology, Sahlgren’s Hospital, University of Gothenburg, S-413 45 Gothenburg, Sweden.

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[3,5,8]. Sheehy [6] finds complications of the disease to be less common in children, but this appears to be related to duration of disease rather than to age. Ossicular chain pathology in children does not vary significantly from what is found in adults and is most pronounced in ears with tensa cholesteatoma [9]. The most common symptoms of cholesteatoma are hearing loss, otorrhea and otalgia [2].

The choice of surgical technic is still controversial. The surgical treatment favoured by Sheehy [61 and Glasscock [3] is canal wall up procedure. Palva [5] uses a canal wall down approach more often and Tos [8], Smyth [7] and Parisier [2] employ both procedures.

We have studied the incidence of cholesteatoma in children living in an urban area of western Sweden. The total popuiation of this area is 470,000 out of which 100,000 are children, 16 years or younger. We also wanted to evaluate the clinical situation and hearing level 4 years or more after surgery.

Method

The charts of all the children undergoing cholesteatoma surgery during the time period from 1977 to 1986 were collected and analyzed. Only those patients were included who had not been exposed to cholesteatoma surgery before, which meant no children with residual or recurrent cholesteatoma.

According to available demographic data for this urban area of western Sweden we have found the average annual population during this time period to be 470,000 out of which 100,000 were children, 16 years or younger.

Almost 100% of the newborn children are included in the Swedish baby health care system with examination at 2, 6 and 10 months, with control of hearing at the last visit. Further examinations are performed at 1.5, 2.5, and 4 years. Routine screening audiometry is performed in 4-year-olds and schoolchildren at the age of 7 and 10 years. This together with the good access of ENT-doctors and the policy among non-otologists to refer children with ear problems in the area studied makes it likely that all the cholesteatoma cases have been traced.

We have also recalled the patients to complete the medical history with the present clinical situation, otomicroscopy and audiometry 4-13 years after primary surgery.

Results

We found 19 patients meeting these criteria; 9 boys and 10 girls with an average of 9 years. The distribution of these cases are most irregular. During the first 2 years, 1977 and 1978, 11 cases were found but in the following 8 years only 8 new cases were identified (Fig. 1). Due to the health care system in Sweden the possibility that some children might have been operated on elsewhere can be ruled out, and we feel that there is an actual change in the incidence in the area studied, though we find the material too small to say that the change is statistically significant.

Cholesteatorna in children 1977-1986

Distribution

6-r r

5 tl I

1

1977 76 79 80 81 82 83 84 85 86 Year

Fig. 1. The incidence of cholesteatoma in 19 children over a IO-year period.

When comparing the two groups, early detected (1977-1978) and later detected (1979-19861, we find no differences concerning age, hearing level preoperatively, ossicular chain pathology and types of perforation and retraction of the ear drum. The duration of the symptoms before operation varied in both groups between 0.5 and 3 years with a median time of 1.5 year. Drainage from the ear was 6 out of 11 vs 2 out of 8.

The majority of the children, 17 out of 19, had episodes of otitis media with intermittent periods of secretory otitis media the year before the discovery of the cholesteatoma. Eight patients had drainage from the ear before operation. Five patients had been treated with ventilation tubes. Only two children had no periods of infection the year before operation. They had dry tensa perforations and were regularly controlled for that. Ten of the 19 cases had a history of otitis media before the age of 2. Radiological investigation before operation showed bone destruction and signs of cholesteatoma in 2 cases.

The size of the cholesteatoma varied from small to larger ones, that is cholesteatoma mass confined to the middle ear to cholesteatoma filling up the whole middle ear, epitympanon and mastoid. Thirteen of the children had perfora- tion of the ear drum. Of these, 7 were attic perforations, 3 posterio-superior tensa perforations, 2 central tensa perforations and one patient had a total tensa perforation. Six children had retraction of the ear drum. Five of the patients had intact ossicular chain but for the rest of the children the cholesteatoma involved the ossicles. Destruction of the arch of the stapes was seen in 5 cases.

Intact wall tympanoplasty was used in 10 cases and canal wall down operation in 9 cases. Residual cholesteatoma was detected in 6 cases and recurrent cholesteatoma in one case. Six of these had an intact wall tympanoplasty and all but one were later reoperated with a canal wall down approach. Thus the number of recurrences was high when intact wall tympanoplasty was used. Five of the recurrences were detected at second look within l-2 years after operation, and in one case it was detected 5 years after primary surgery. The results in our study favour the canal wall down operation.

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dB HL Before After Follow up

20

30

40

50

60

_ Arch of stapes intact (14)

-+-__-j( Arch of stapes destroyed (5)

Fig. 2. The hearing impairment before and after operation and at follow-up in 19 patients.

The hearing has been evaluated in all 19 cases pre- and postoperatively with pure tone audiometry (PTA), and is based on the mean value of the frequency range 500-2000 Hz. At the late follow-up in 1990, speech reception threshold has been estimated for the patients. The hearing varied from 10 dB to 55 dB before operation and from 8 dB to 55 dB after operation. The hearing was best when the ossicular chain was unbroken and worst in those 5 cases when the crura of the stapes were destroyed (Fig. 2). Ossicular reconstruction has been performed in 3 cases resulting in improved hearing. For the rest of the patients not subjected to ossicular reconstruction the hearing has become impaired in 4 cases and for the remainder it is unchanged. Five of the 19 patients are today completely satisfied with their hearing situation. The remainder of the patients experience more or less pronounced difficulties in different situations. Three patients regularly use a hearing aid. None of the patients has now any obvious problem with drainage from the ear. The median follow-up time for the children has been 10.5 years within a range of 4-13 years.

Discussion

The diagnosis of pediatric cholesteatoma is often difficult and there are several reasons for this. Many times there is no pain or drainage associated with the cholesteatoma and the hearing loss, particularly when only one ear is involved, may not be detected until a routine audiogram is administrated. Some children have

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small and tortuous ear canals and because of their unwillingness to co-operate examination is often difficult. Attic cholesteatoma is particularly troublesome in that it often manifests itself only as a small crust on the Shrapnel13 membrane.

In the mid 70’s new technics and routines were introduced in our area for examining children with ear problems, especially those with secretory otitis media. Our routine screening program with audiometry in 4-year-olds and schoolchildren was improved by an increased use of tympanometry as well as a more general use of otomicroscopy.

We believe that it is due to the good access of ENT-doctors and a policy among non-otologists to refer small children to an otologist, that we found in this study a low incidence of 2 children with cholesteatoma out of 100,000 children each year. In the northernmost county of Sweden, where for many years the number of otologists has been very low, the incidence reported is 4 times as high [l]. From Denmark, Tos [9] has reported an incidence of 15 children out of 100,000 children each year. From Finland, Karma et al. [4] found an annual incidence of 4.5 children out of 100,000.

The decline in the cholesteatoma incidence recorded is very satisfying. It should be remembered, however, that a cholesteatoma is a great trauma for the child. It means surgery, in some cases also reoperations, and often a considerable hearing loss, with a need for hearing aid in some cases. Early detection and treatment of children at risk of developing cholesteatoma seems to be the best way to reduce the cholesteatoma incidence.

In accordance with Tos [9] we believe in the retraction theory which favours the assumption that the majority of cholesteatomas, in children as well as in adults, results from secretory otitis media, which causes atrophy of the tympanic mem- brane and retraction pockets which at a later stage develops into cholesteatoma.

Conclusion

Our study reveals a decrease in the incidence of cholesteatoma over a lo-year period. A possible explanation is the more frequent use of tympanometry and otomicroscopy by all ENT-doctors and regular examination and treatment of children with secretory otitis media.

References

1 Bunne, M. and Raivo M., Pitfalls in diagnosis and treatment of cholesteatoma in children. In M. Tos, J. Thomsen and E. Peitersen (Eds.), Cholesteatoma and Mastoid Surgery, Kugler Publications. Amsterdam, 1989, pp. 651-656.

2 Edelstein, D.R., Parisier, SC., Chute, P., Ahuja, G.S., Jurabe, C., Wenig, S. and Kaye, SM.. Cholesteatoma in pediatric age group, Ann. Otol. Rhinol. Laryngol., 97 (1988) 23-29.

3 Glasscock, M.E., Dickins, J.R.E. and Wiet, R.. Choleteatoma in children, Laryngoscope, 91 (1981) 1743-1753.

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4 Karma, P., SipilB, M., Pukander, J. and PHrIII, M., Occurrence of cholesteatoma and secretory otitis media in children. In M. Tos, J. Thomsen and E. Peitersen (Eds.), Cholesteatoma and Mastoid Surgery, Kugler Publ. Amsterdam, 1989, pp. 335-338.

5 Palva, A., Karma, P. and Karja, J. Cholesteatoma in children, Arch. Otolaryngol., 103 (1977) 74-77. 6 Sheehy, J.L., Cholesteatoma surgery in children, Am. J. Otol., 6 (1985) 170-172. 7 Smyth, G.D.L., Postoperative cholesteatoma in combined approach tympanoplasty: fifteen year

report on tympanoplasty, part 1, J. Laryngol. Otol., 90 (1976) 597-621. 8 Tos, M., Treatment of cholesteatoma in children, a long-term study of results, Am. J. Otol., 4 (1983)

189-197. 9 Tos, M., Incidence, Etiology and Pathogenesis of cholesteatoma in children. In C.R. Pfaltz (Ed.),

Advances in Oto-Rhino-Laryngology, Vol. 40, Karger Publications, Basel, 1988, pp. 110-117.