FUNCTIONAL SURGERY FOR PEDIATRIC CHOLESTEATOMA: A 25-YEAR LONGITUDINAL STUDY

Chin-Lung Kuo, An-Suey Shiao, Wen-Huei Liao, Chiang-Feng Lien

Department of Otorhinolaryngology-Head and Neck Surgery, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan

Introduction

There has been a trend of less radical surgery with functional preservation that was being applied in many fields. For example, functional endoscopic sinus surgery has been applied in treating chronic rhinosinusitis, and transoral laser microsurgery was appliedfor laryngeal and hypopharyngeal cancers so as to avoid total laryngectomy.

Since 1986, Dr. Lien has performed a functional surgery for pediatric cholesteatoma: tailor-made tympano-mastoidectomy with cartilage reconstruction (TTCR). This technique emphasizes the importance of preserving uninvolved, functional mastoid air cells, and anatomical reconstruction with cartilage, which may facilitate mastoid aeration and avoid cavity problems.

The purpose of the study was to evaluate the long-term outcomes after TTCR for pediatric cholesteatoma and to analyze the impact of the applied statistical method on the recurrence rate.

Methods

From 1986 to 2011, we retrospectively screened a consecutive series of children (18 years or younger) with acquired cholesteatomas after primary TTCR.

The pre-operative conditions were recorded according to the CAO staging system of cholesteatoma proposed by Dr. Lien in 1985.1

Outcome assessments

The medical records of all patients were analyzed for the last otologic examination. Data were collected on sex, ear side, age of surgery, operative findings (i.e., cholesteatoma extent, degree of atelectasis, ossicular condition, labyrinthine fistula, and facial nerve dehiscence caused by cholesteatoma), recidivistic disease, and complications.

The anatomical outcomes were determined by the cumulative recurrence rate and recurrence-free survival rate (percentage of ears free from recurrence) at five, ten, 15 and 20 years after the initial surgery.

Surgical techniques

The new surgical technique employed was one-stage TTCR via an anterior approach. An endaural incision was made to expose the external auditory canal (EAC) and temporalis fascia. The fascia was harvested as a graft. After elevation of the tympanomeatal flap from the posterior canal wall, the EAC was enlarged and the scutum was removed, giving direct access to the cholesteatoma.

Retrograde mastoidectomy was applied to remove the cholesteatoma, which extended along the route of involvement, thus creating an atticotomy, attico-antrectomy or attico-antro-mastoidectomy open cavity (Fig. 1). TTCR emphasizes the importance of preserving uninvolved functional middle-ear structures and mastoid air cells, which facilitates post-operative middle-ear ventilation.

Conchal cartilage was harvested to reconstruct the posterior canal wall and maintain the EAC anatomy, avoiding post-operative cavity problems.

Tympanoplasty was then performed to restore hearing.

image

Fig. 1. Progressive retrograde mastoidectomy is individualized by the extent of the cholesteatoma, thus creating three types of tailor-made tympanomastoidectomy with cartilage reconstruction. c: obliterated cartilage in the tailor-made open cavity.

Statistical analysis

The cumulative recurrence rate was calculated by summation of the recurrence rates obtained for each observation year.

However, because the length of the post-operative observation period varied, the true percentage of ears free from recurrence may have been overestimated using the standard rate calculation.2,3 Event-free survivals were thus determined using the Kaplan-Meier survival analysis.

Statistical comparisons and descriptive statistics were conducted using a commercially available software package, SPSS version 18.0 (SPSS, Inc., Chicago, IL, USA).

Results

The patient characteristics are shown in Table 1. A total of 59 operations were performed on 57 patients. Two patients experienced bilateral cholesteatomas. The mean age was 10.7 ± 4.5 years. The mean follow-up period was 14.1 ± 7.1 years, and 49 (83%) operations had a follow-up period of five years or longer. Dryness, self-cleaning and water-resistance were observed in ears without recidivism.

Cholesteatoma recidivism was observed in seven ears (five recurrent and two residual diseases). The mean detection time was 10.4 years, range 1.9–17.2 years. Five of seven ears (71.4%) with recidivism were detected more than ten years after surgery.

Cumulative recidivism rates calculated by different methods are shown in Table 2. The recidivism-free probabilities at five, ten, 15 and 20 years were 98.2%, 96.2%, 84.3% and 79.6%, respectively. Therefore, the recidivism rates at five, ten, 15 and 20 years as calculated by the Kaplan-Meier method were 1.8%, 3.8%, 15.7% and 20.4%, respectively. The cumulative recidivism rates as calculated by the standard rate method at five, ten, 15 and 20 years were 1.7%, 3.4%, 10.2% and 11.9%, respectively. The recidivism rates at five, ten, 15 and 20 years as calculated by the standard rate method were underestimated compared with those by Kaplan-Meier analysis. Besides, the difference in recidivism rates between the two calculation methods increased with the follow-up time, from 0.1% to 8.5% between five and 20 years of follow up.

Table 1. Patient characteristics (n =59).

Variables

No. (%)

Sex

Male

33 (55.9%)

Female

26 (44.1%)

Ear

Right

37 (62.7%)

Left

22 (37.3%)

Age of surgery (years)

10.69 ± 4.48

Follow-up period (years)

14.10 ± 7.10

Stage of CAO system

Stage I

13 (22.0%)

Stage II

12 (20.3%)

Stage III

34 (57.6%)

Intact ossicular chain

11 (18.6%)

Labyrinthine fistula

4 (6.8%)

Facial nerve dehiscence

14 (23.7%)

Table 2. Cumulative recidivism rates calculated by different methods.

image

Conclusions

The long-term outcomes in this study show that the functional surgery, TTCR, is an alternative and valid therapeutic option for children with cholesteatoma, allowing for higher rates of recurrence-free survival and cavity problem control, facilitating easy postoperative care for children.

The Kaplan-Meier survival analysis method should be used when discussing recidivism rates. The standard rate calculation method should be used only in cases where there are no censored data.

Due to late recurrences, the importance of long-term follow up cannot be overemphasized. We recommend that parents ensure that their children receive follow up periodically until they reach adulthood.

References

1.Lien CF. Staging of cholesteatoma. Amsterdam: Elsevier Science Publishers BV, 1985

2.Parisier SC, Hanson MB, Han JC, Cohen AJ, Selkin BA. Pediatric cholesteatoma: an individualized, single-stage approach. Oto-laryngol Head Neck Surg 115:107–114, 1996

3.Stangerup SE, Drozdziewicz D, Tos M, Hougaard-Jensen A. Recurrence of attic cholesteatoma: different methods of estimating recurrence rates. Otolaryngol Head Neck Surg 123:283–287, 2000


Address for correspondence: Dr. Chiang-Feng Lien, Department of Otorhinolaryngology-Head and Neck Surgery, Taipei Veterans General Hospital, No. 201, Section 2, Shih-Pai Road, Taipei 112, Taiwan. drtgud@gmail.com

Cholesteatoma and Ear Surgery – An Update, pp. 225–227

Edited by Haruo Takahashi

2013 © Kugler Publications, Amsterdam, The Netherlands