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Congenital middle ear cholesteatomas in children: our experience in 34 cases
Vincent Darrouzet1, Jean-Yves Duclos, Didier Portmann
1Department of Otorhinolaryngology, University Hospital of Bordeaux, France. vincent.darrouzet@chu-bordeaux.fr
Insights
Congenital cholesteatoma in children has a higher residual disease rate than acquired forms, but offers better hearing outcomes. Staged closed technique (CT) surgery is recommended for optimal treatment.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Medical Research
Background:
- Cholesteatoma in children can be congenital or acquired, presenting distinct clinical challenges.
- Understanding differences in treatment outcomes is crucial for pediatric otologic care.
Purpose of the Study:
- To compare clinical data, residual disease rates, and functional hearing results between congenital and acquired cholesteatomas in pediatric patients.
- To evaluate the effectiveness of different surgical techniques for cholesteatoma removal in children.
Main Methods:
- Retrospective analysis of 34 congenital cholesteatomas from a series of 215 pediatric cholesteatomas over 10 years.
- Surgical interventions included closed technique (CT), open technique tympanoplasty (TOT), radical mastoidectomy (RM), and Rambo's technique.
- Outcomes assessed included surgical findings, residual lesion rates, and postoperative hearing (pure tone average, air-bone gap, speech reception threshold).
Main Results:
- Congenital cholesteatomas showed a higher residual lesion rate compared to acquired forms.
- Hearing results were significantly better for congenital cholesteatomas.
- Mean postoperative air-bone gap was 21 dB, with 66% achieving a speech reception threshold < 30 dB HL.
- Staged closed technique (CT) with a planned second look demonstrated a residual lesion rate of 29.2%.
Conclusions:
- Staged closed technique (CT) is often the preferred surgical approach for pediatric cholesteatomas, particularly those in well-pneumatized mastoids.
- While congenital cholesteatomas may have higher recurrence rates, surgical interventions can yield favorable hearing outcomes.
- Careful surgical planning and follow-up are essential for managing pediatric cholesteatomas.
Objectives:
To evaluate clinical data, extensions, residual disease rate, and functional results in cases of congenital cholesteatoma in pediatric patients compared with cases of acquired forms.
Study Design And Setting:
In a retrospective study conducted at a single tertiary care center over a decade, 34 congenital cholesteatomas (mean patient age, 6.6 years) isolated from a series of 215 cholesteatomas in children were treated surgically and followed up for an average of 83 months. Surgical treatment consisted of the closed technique (CT), open technique tympanoplasty in CT [TOT], radical mastoidectomy (RM), or Rambo's technique. The main outcome measures were surgical findings, residual lesion rate, and hearing assessment.
Results:
The first surgical procedure was CT in 85.3%, TOT in 8.8%, RM in 2.9%, and Rambo's technique in 2.9% of patients. Two, 3, and 4 operations were necessary in 76.5%, 11.4%, and 11.4% of children, respectively. Residual lesions were observed in 34.6% of those multi-operated patients (7 CT and 2 TOT), 24.1% in those treated with CT, and 29.2% in those treated with CT with a planned second look. Mean postoperative pure tone average and air-bone gap were 26 and 21 dB, respectively. A speech reception threshold of less than 30 dB HL was achieved in 66% of patients. A total of 26 ossiculoplasties had to be performed (8 partial and 18 total); the mean postoperative air-bone gap was lower after the former (15 dB) than after the latter (22 dB). A high-frequency pure-tone hearing loss of more than 10 dB was uncommon (5.8%). Unsurprisingly, the residual lesion rate seems to be higher for congenital than for acquired cholesteatomas, but hearing results are significantly better.
Conclusion:
In most cases, staged CT appears to be the best technique to treat these lesions, which often develop in a well-pneumatized mastoid.