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      Congenital middle ear
    cholesteatomas in children
A Abbas, R Nicollas, I Sudre, H Belaïche, S Roman, JM Triglia


                                              Dr Ali ABBAS
                                        Pediatric ENT department
                                        Timone Children Hospital
                                            Marseille France
                                                 20/06/2006
Why?


   Problem: early diagnosis
   Comparative study with acquired cholesteatoma in
    children
   Objective: to determine criterias allowing earlier diagnosis
Material and methods
 Monocentric and comparative prospective study from 1994 to 2005,
 n = 322 cases of cholesteatoma in children
 Mean follow-up = 48 months
 Canal wall up procedure




Congenital cholesteatoma               Acquired cholesteatoma
         n=61                                  n=261

4 bilateral cases                     14 bilateral cases
Mean age: 6 years [1,1 to 13,9]       Mean age: 10 years [3,4 to 18]
Gender: 59% boys                      gender: 62% boys
Results
                         Clinical    n=61
                          otalgia
                                           non symptomatic
serous otitis media            13%
                  9%                       31%
                       24%
                                     23%


              deafness                      otorrhea
Results
                                Initial location n=61
 anterior superior                     posterior superior
     quadrant                              quadrant

                     83 %       31 %

anterior inferior                        posterior inferior
   quadrant                                  quadrant

                      3%      3%
Surgical results
                       Procedure n=61
 limited pattern (anterior superior): 23%             exploratory
tympanotomy (simple removal, better prognosis)


 extended pattern (posterior & diffuse): 77%            CWU
tympanomastoidectomy (2nd look reexploration, ossiculoplasty)


            Recurrence rate: 20,3% of cases
Comparative study (κ     2   Student)



     Congenital (n=61)    Acquired (n=261)
Asymptomatic        31%      8%
Deafness            24%      24%
Otitis              22%      -
Otorrhea            23%      80%



                                   p<0,05
Anatomical presentation

                 Congenital (n = 61)   Acquired (n = 261)

“cystic” closed pattern      36%             54%
“infiltrate”opened pattern   64%             46%


without ossicle lysis        63%             25%
incus lysis                  64%             68%
stapes lysis                 40%             31%


                                           p<0,05
Contralateral ear

             Congenital (n=61)   Acquired (n=261)

Normal                    84%           56%
SOM                       12%           13%
Chronic otitis             4%           31%


                                       p<0,05
Postoperative hearing assessments


      Congenital (n=61)                     Acquired (n=261)

Preoperative ABG (dB):       28 [26 à 59]     27 [7 à 56]

Postoperative ABG (dB):      15 [0 à 47]      17 [0 à 60]

Preoperative mean AC (dB):           38       35


                                                   p<0,05
Discussion
   Levenson’s criterias:
     – CC children with AOM
     – under-estimated incidence: perforation post CC

• Location
Series           Year     Number of   Antero-mallear    Postero-
                            cases          (%)         mallear(%)
Parisier         1989        40           67,5            15
McGill           1991        41            41              0
Postic           2002        172           82             49
Nelson           2002        119          85,1            77
Our study        2006        61            83             31
Conclusion

   location relates to postoperative hearing results
    and the likelihood of residual disease
   earlier diagnosis = better surgical result
   Large increase in reports of CC

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Congenital Cholesteatoma ESPO Paris

  • 1. www.orl-marseille.com Congenital middle ear cholesteatomas in children A Abbas, R Nicollas, I Sudre, H Belaïche, S Roman, JM Triglia Dr Ali ABBAS Pediatric ENT department Timone Children Hospital Marseille France 20/06/2006
  • 2. Why?  Problem: early diagnosis  Comparative study with acquired cholesteatoma in children  Objective: to determine criterias allowing earlier diagnosis
  • 3. Material and methods  Monocentric and comparative prospective study from 1994 to 2005,  n = 322 cases of cholesteatoma in children  Mean follow-up = 48 months  Canal wall up procedure Congenital cholesteatoma Acquired cholesteatoma n=61 n=261 4 bilateral cases 14 bilateral cases Mean age: 6 years [1,1 to 13,9] Mean age: 10 years [3,4 to 18] Gender: 59% boys gender: 62% boys
  • 4. Results Clinical n=61 otalgia non symptomatic serous otitis media 13% 9% 31% 24% 23% deafness otorrhea
  • 5. Results Initial location n=61 anterior superior posterior superior quadrant quadrant 83 % 31 % anterior inferior posterior inferior quadrant quadrant 3% 3%
  • 6. Surgical results Procedure n=61  limited pattern (anterior superior): 23% exploratory tympanotomy (simple removal, better prognosis)  extended pattern (posterior & diffuse): 77% CWU tympanomastoidectomy (2nd look reexploration, ossiculoplasty) Recurrence rate: 20,3% of cases
  • 7. Comparative study (κ 2 Student) Congenital (n=61) Acquired (n=261) Asymptomatic 31% 8% Deafness 24% 24% Otitis 22% - Otorrhea 23% 80% p<0,05
  • 8. Anatomical presentation Congenital (n = 61) Acquired (n = 261) “cystic” closed pattern 36% 54% “infiltrate”opened pattern 64% 46% without ossicle lysis 63% 25% incus lysis 64% 68% stapes lysis 40% 31% p<0,05
  • 9. Contralateral ear Congenital (n=61) Acquired (n=261) Normal 84% 56% SOM 12% 13% Chronic otitis 4% 31% p<0,05
  • 10. Postoperative hearing assessments Congenital (n=61) Acquired (n=261) Preoperative ABG (dB): 28 [26 à 59] 27 [7 à 56] Postoperative ABG (dB): 15 [0 à 47] 17 [0 à 60] Preoperative mean AC (dB): 38 35 p<0,05
  • 11. Discussion  Levenson’s criterias: – CC children with AOM – under-estimated incidence: perforation post CC • Location Series Year Number of Antero-mallear Postero- cases (%) mallear(%) Parisier 1989 40 67,5 15 McGill 1991 41 41 0 Postic 2002 172 82 49 Nelson 2002 119 85,1 77 Our study 2006 61 83 31
  • 12. Conclusion  location relates to postoperative hearing results and the likelihood of residual disease  earlier diagnosis = better surgical result  Large increase in reports of CC