Acta Scientific Orthopaedics (ISSN: 2581-8635)

Research Article Volume 5 Issue 5

Surgical Treatment Of Congenital Cholesteatoma of Middle Ear at Children

Ivan Baljošević1*, Ognjen Milicevic2 and Stefan Popovic1

1ENT Department, Mother and Child Institute, Belgrade, Faculty of Dentistry, Pancevo, Serbia
2Department of Medical Statistics and Informatics, Medical Faculty University of Belgrade, Belgrade, Serbia

*Corresponding Author: Ivan Baljošević, ENT Department, Mother and Child Institute, Belgrade, Faculty of Dentistry, Pancevo, Serbia.

Received: February 14, 2022; Published: April 11, 2022

Abstract

Objectives: Congenital cholesteatoma presents pathological lesion that develops behind intact membrane tympani. Objective of this paper is to present our experience in diagnostics and treatment of congenital cholesteatoma at children.

Material and Methods: Our study represents a retrospective review of 17 children who underwent surgery for congenital cholesteatoma from 2012. to 2019. at the ORL Department, Mother and Child Health Care Institute, Belgrade, Serbia. All patients had congenital cholesteatoma present on one ear. Diagnoses were made from between 3 months to one year after presentation (an average of 6.3 months).

Basic criteria in diagnosis of congenital cholesteatoma were presence of white mass behind the intact timpani membrane with no data on otorrhea, perforation of membrane timpani or surgical interventions. Staging of the cholesteatomas was based on the classification suggested by Potsic.

Results: Congenital cholesteatoma confined to the middle ear cavity was present in 16 (94%) patients, the majority of whom had involvement of multiple quadrants (9 patients or 57%), in 4 (25%) cases CC was localized to the posterior quadrants, in 2 (12%) to the anterior quadrants and in one (6%) case to the anterosuperior and posterosuperior quadrants. One case involved congenital cholesteatoma confined to the mastoid cavity. Preoperative contralateral middle ear disease was absent in 12 (70%) cases, whereas secretory otitis media (SOM) was identified in the contralateral ear in 3 (18%) cases and adhesive otitis in 2 (12%) cases. The following surgical interventions were performed: tympanoplasty type I in 3 (17%) patients with congenital cholesteatoma localized to the anterior quadrants, radical modified tympanomastoidectomy (canal wall up) and tympanoplasty type II in 5 (30%) patients, canal wall down mastoidectomy and tympanoplasty type III in 8 (47%) and simple (cortical) mastoidectomy in 1 (6%) patient (where the cholesteatoma was confined to the mastoid cavity). All patients were followed up 24 ± 6,4 months postoperatively. Recurrent cholesteatoma was diagnosed in 2 (12%) patients, one in a patient who had CWU and one in a patient who had CWD mastoidectomy performed. In both cases a small aplastic poorly pneumatized mastoid was encountered during surgery and both patients had concomitant contralateral middle ear disease (SOM and adhesive otitis media respectively).

Conclusions: Congenital cholesteatoma is characterized by insidious onset with predominantly ipsilateral CHL, which is why in many cases patients are erroneously treated for otitis media with effusion prior to establishment of correct diagnosis. Furthermore, the insidious nature of disease calls for a high index of suspicion in ORL specialists and indeed pediatricians performing newborn hearing screening and hearing tests in preschool children as CCs are most commonly diagnosed by that age. Disease stage is a significant risk factor for recurrence.

Keywords: Congenital Cholesteatoma; Stage; Surgical Procedures

References

  1. Derlacki EL and Clemis GL. “Congenital cholesteatoma of the middle ear and mastoid”. Annals of Otology, Rhinology and Laryngology 74 (1965): 706-727.
  2. Levenson MJ., et al. “A review of twenty congenital cholesteatomas a middle ear in children”. Otolaryngology–Head and Neck Surgery 15 (1986): 169-174.
  3. Potsic W., et al. “A staging system for congenital cholesteatoma”. Archives of Otolaryngology--Head and Neck Surgery 128 (2002): 1009-1012.
  4. Tos M. “A new pathogenesis of mesotympanic (congenital) cholesteatoma”. Laryngoscope 110 (2000): 1890-1897.
  5. Mcgill T J., et al. “Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report”. Laryngoscope 101 (1991): 606-613.
  6. Teed RW. “Cholesteatoma verum tympani its relationship to the first epibranchial placode”. Archives of Otolaryngology 24 (1936): 455-474.
  7. Lee ST., et al. “The epidermoid formation and its affinity to congenital cholesteatoma”. Clinical Otolaryngology 23 (1998): 449-454.
  8. Reudi L. “Cholesteatoma formation in the middle ear in animal experiments”. Acta Otolaryngology (Stockh) 5 (1959)0:232-242.
  9. Sade J., et al. “The metaplastic and congenital origin of cholesteatoma”. Acta Otolaryngology (Stockh) 96 (1983): 119-129.
  10. Aimi K. “Role of tympanic ring in the pathogenesis of congenital cholesteatoma”. Laryngoscope 93 (1983): 1140-1146.
  11. Northrop C., et al. “Histological observations of amniotic fluid cellular content in the ear of neonates and infants”. International Journal of Pediatric Otorhinolaryngology 11 (1986): 113-127.
  12. Darrouzet V., et al. “Congenital middle ear cholesteatoma in children: our experience in 34 cases”. Otolaryngology–Head and Neck Surgery 126 (2002): 34-40.
  13. El-Bitar M., et al. “Congenital middle ear cholesteatoma: need for early recognition-role of computed tomography scan”. International Journal of Pediatric Otorhinolaryngology 67 (2003): 231.
  14. Yung M., et al. “EAONO/JOS joint consensus statements on the definitions, classification and staging of middle ear cholesteatoma”. Journal of International Advanced Otology1 (2017): 1-8.
  15. McCabe R., et al. “The Endoscopic Management of Congenital Cholesteatoma”. Otolaryngologic Clinics of North America 54 (2021): 111-123.
  16. Pasanis E., et al. “Congenital cholesteatoma of tympanic membrane”. International Journal of Pediatric Otorhinolaryngology 2 (2001): 167-171.
  17. Yalcin S., et al. “First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia”. International Journal of Pediatric Otorhinolaryngology 67 (2003): 811-814.
  18. Hao J., et al. “The Significance of Staging in the Treatment of Congenital Cholesteatoma in Children”. Ear, Nose and Throat Journal10 (2021): 1125S-1131S.
  19. Park J., et al. “Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma”. Clinical and Experimental Otorhinolaryngology4 (2018): 233-221.
  20. Kazahaya K and Potsic W. “Congenital cholesteatoma”. Current Opinion in Otolaryngology and Head and Neck Surgery 12 (2004): 398-403.
  21. Cvorovic Lj., et al. “Congenital cholesteatoma of mastoid origin - A multicenter case series”. Vojnosanitetski pregled7 (2017): 619-622.
  22. Iino Y., et al. “Mastoid pneumatization in children with congenital cholesteatoma: an aspect of the formation of open-type and closed type cholesteatoma”. The Laryngoscope7 (1998): 1071-1076.
  23. Leong HK., et al. “Congenital cholesteatoma of the middle ear and mastoid: a report of five cases”. Revue de Laryngologie - Otologie - Rhinologie (Bord) 113 (1992): 41-45.
  24. Kamarkar S., et al. “Congenital cholesteatomas of the middle ear”. American Journal of Otology 17 (1996): 288-292.
  25. Song S I., et al. “Clinical Characteristics and Treatment Outcomes of Congenital Cholesteatoma”. Journal of International Advanced Otology3 (2019): 386-390.

Citation

Citation: Ivan Baljošević., et al. “Surgical Treatment Of Congenital Cholesteatoma of Middle Ear at Children".Acta Scientific Orthopaedics 5.5 (2022): 40-46.

Copyright

Copyright: © 2022 Ivan Baljošević., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.




Metrics

Acceptance rate33%
Acceptance to publication20-30 days

Indexed In



News and Events


  • Certification for Review
    Acta Scientific certifies the Editors/reviewers for their review done towards the assigned articles of the respective journals.
  • Submission Timeline for Upcoming Issue
    The last date for submission of articles for regular Issues is December 25, 2024.
  • Publication Certificate
    Authors will be issued a "Publication Certificate" as a mark of appreciation for publishing their work.
  • Best Article of the Issue
    The Editors will elect one Best Article after each issue release. The authors of this article will be provided with a certificate of "Best Article of the Issue"

Contact US