Surgery for Pediatric Cholesteatoma 

  • Author: Peter S Roland, MD; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP   more...
 
Updated: Mar 25, 2010
 

Background

For decades, cholesteatoma has been recognized as a destructive lesion of the skull base that can erode and destroy important structures within the temporal bone. Its capacity for CNS complications, such as brain abscess and meningitis, make it a potentially fatal lesion.

History

Although Cruveilhier first described cholesteatoma in 1829, Müller first named the lesion in 1858. Throughout the early half of the 20th century, cholesteatoma was managed by exteriorization. The mastoid air cells were exenterated, the posterior wall of the external auditory canal was removed, and the opening into the resulting cavity was enlarged to ensure adequate air exchange and to make visual inspection simple.

During the 1950s and 1960s, the House Otologic Group developed a new approach. The group attempted to leave the basic underlying anatomic structure of the ear and temporal bone intact, principally by preserving the canal wall. These aggressive attempts to conserve the normal anatomy of the ear created great controversy. Surgeons tended to align themselves with either the old "canal wall down" (ie, open cavity) philosophy[1] or with the new "canal wall up" (ie, closed cavity) philosophy.[2] Throughout the last 2 decades, most otologic surgeons have migrated to an intermediate position. In general, most otologic surgeons in the United States now perform both techniques, selecting a particular operation based on the individual circumstances of each patient.

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Pathophysiology

A cholesteatoma consists of squamous epithelium that is trapped in the skull base. Squamous epithelium trapped within the temporal bone (ie, middle ear or mastoid) can expand only at the expense of the bone that surrounds and contains it. Consequently, complications associated with a growing cholesteatoma can include injury to any of the structures normally found within the temporal bone. Occasionally, a cholesteatoma escapes the confines of the temporal bone and skull base. Extratemporal complications can occur in the neck, CNS, or both. When a cholesteatoma within the cranium grows enough to produce mass effect, brain dysfunction can develop.

Bony erosion occurs by 2 principal mechanisms, as follows:

  • Pressure effects produce bony remodeling.
  • Enzymatic activity at the margin of the cholesteatoma enhances osteoclastic activity, which greatly increases the speed of bone erosion. These osteolytic enzymes appear to increase when a cholesteatoma becomes infected.
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Epidemiology

Frequency

United States

The incidence is unknown, but a cholesteatoma is a relatively common reason for otologic surgery.

Mortality/Morbidity

Death from intracranial complications of cholesteatoma is now uncommon because of earlier recognition, timely surgical intervention, and supportive antibiotic therapy. Cholesteatoma remains a relatively common cause of permanent, moderate, conductive hearing loss.

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Contributor Information and Disclosures
Author

Peter S Roland, MD  Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development

Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Alcon Labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear Corp Honoraria Board membership; Med El Corp travel grants Consulting

Specialty Editor Board

Orval Brown, MD  Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas

Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

John E McClay, MD  Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Dallas, University of Texas Southwestern Medical School

John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Glenn C Isaacson, MD, FACS, FAAP  Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine

Glenn C Isaacson, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Covidien Honoraria Consulting

References

  1. Roland PS, Meyerhoff WL. Open-cavity tympanomastoidectomy. Otolaryngol Clin North Am. Jun 1999;32(3):525-46. [Medline].

  2. Graham MD, Delap TG, Goldsmith MM. Closed tympanomastoidectomy. Otolaryngol Clin North Am. Jun 1999;32(3):547-54. [Medline].

  3. Manolidis S, Kutz JW Jr. Diagnosis and management of lateral sinus thrombosis. Otol Neurotol. Sep 2005;26(5):1045-51. [Medline].

  4. Kazahaya K, Potsic WP. Congenital cholesteatoma. Curr Opin Otolaryngol Head Neck Surg. Oct 2004;12(5):398-403. [Medline].

  5. Potsic WP, Korman SB, Samadi DS, Wetmore RF. Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia. Otolaryngol Head Neck Surg. Apr 2002;126(4):409-14. [Medline].

  6. Dawes PJ, Leaper M. Paediatric small cavity mastoid surgery: second look tympanotomy. Int J Pediatr Otorhinolaryngol. Feb 2004;68(2):143-8. [Medline].

  7. De la Cruz A, Fayad JN. Detection and management of childhood cholesteatoma. Pediatr Ann. Jun 1999;28(6):370-3. [Medline].

  8. Dornhoffer JL, Colvin GB, North P. Evidence of residual disease in ossicles of patients undergoing cholesteatoma removal. Acta Otolaryngol. Jan 1999;119(1):89-92. [Medline].

  9. Busaba NY. Clinical presentation and management of labyrinthine fistula caused by chronic otitis media. Ann Otol Rhinol Laryngol. May 1999;108(5):435-9. [Medline].

  10. Anderson J, Caye-Thomasen P, Tos M. A comparison of cartilage palisades and fascia in tympanoplasty after surgery for sinus or tensa retraction cholesteatoma in children. Otol Neurotol. Nov 2004;25(6):856-63. [Medline].

  11. Bennett M, Warren F, Jackson GC, Kaylie D. Congenital cholesteatoma: theories, facts, and 53 patients. Otolaryngol Clin North Am. Dec 2006;39(6):1081-94. [Medline].

  12. Chadha NK, Jardine A, Owens D, et al. A multivariate analysis of the factors predicting hearing outcome after surgery for cholesteatoma in children. J Laryngol Otol. Nov 2006;120(11):908-13. [Medline].

  13. Golz A, Goldenberg D, Netzer A, et al. Cholesteatomas associated with ventilation tube insertion. Arch Otolaryngol Head Neck Surg. Jul 1999;125(7):754-7. [Medline].

  14. Kemppainen HO, Puhakka HJ, Laippala PJ, et al. Epidemiology and aetiology of middle ear cholesteatoma. Acta Otolaryngol. 1999;119(5):568-72. [Medline].

  15. Migirov L, Duvdevani S, Kronenberg J. Otogenic intracranial complications: a review of 28 cases. Acta Otolaryngol. Aug 2005;125(8):819-22. [Medline].

  16. Ottaviani F, Neglia CB, Berti E. Cytokines and adhesion molecules in middle ear cholesteatoma. A role in epithelial growth?. Acta Otolaryngol. 1999;119(4):462-7. [Medline].

  17. Semaan MT, Megerian CA. The pathophysiology of cholesteatoma. Otolaryngol Clin North Am. Dec 2006;39(6):1143-59. [Medline].

  18. Thompson JW. Cholesteatomas. Pediatr Rev. Apr 1999;20(4):134-6. [Medline].

  19. Tierney PA, Pracy P, Blaney SP, Bowdler DA. An assessment of the value of the preoperative computed tomography scans prior to otoendoscopic 'second look' in intact canal wall mastoid surgery. Clin Otolaryngol Allied Sci. Aug 1999;24(4):274-6. [Medline].

 
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A typical audiogram demonstrating bilateral conductive hearing loss, which may be observed in an individual with a cholesteatoma.
The photo exhibits a large meatoplasty performed as part of an open cavity (canal wall down) mastoidectomy. A similar meatoplasty usually is necessary if a clean, dry, problem-free cavity is to be maintained.
An unenhanced CT scan demonstrating that the posterior canal wall has been eroded and the external auditory canal has filled with cholesteatoma, pus, and debris. Surprisingly, the middle ear appears relatively free of disease, a characteristic of primary acquired cholesteatomas.
 
 
 
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