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(Pediatrics in Review. 2008;29:330-331.)
© 2008 American Academy of Pediatrics
In Brief |
| The first 20% of the full text of this article appears below. |
Congenital Cholesteatoma: Theories, Facts, and 53 Patients. Bennett M, Warren F, Jackson GC, Kaylie D. Otolaryngol Clin North Am.2006 ;39 :1081 –1094[CrossRef][Medline] The Pathophysiology of Cholesteatoma. Semaan MT, Megerian CA. Otolaryngol Clin North Am.2006 ;39 :1143 –1159[CrossRef][Medline] Complications of Chronic Otitis Media and Cholesteatoma. Smith JA, Danner CJ. Otolaryngol Clin North Am.2006 ;39 :1237 –1255[CrossRef][Medline] Chronic Otitis Media, Mastoiditis, and Petrositis. Chole RA, Sudhoff HH. In: Cumming CW, Schuller DE, Thomas JR, Robbins KT, Harker LA, eds. Otolaryngology: Head and Neck Surgery. 4th ed. Vol 3. Philadelphia, Pa: Elsevier Mosby;2005
The first report describing what we know as a cholesteatoma was by Cruveilhier in 1829, but the term itself, which translates to "pearl-like tumor," was not coined until 1838 by Müller. Today, cholesteatoma is defined as an abnormal accumulation of epithelial debris within the middle ear that causes destructive changes as it expands and erodes into adjacent structures. With an incidence estimated at 3 per 100,000 children, cholesteatoma can present at any age. Early recognition and treatment are essential to avoid otologic or intracranial complications.
Cholesteatomas are classified as congenital or acquired. Most are acquired, with only about 2% to 5% being congenital. Congenital cholesteatomas are believed to arise from embryologic epithelial rests within the middle ear that persist beyond gestation. Acquired cholesteatomas are divided into primary and secondary subgroups. Primary acquired cholesteatomas are believed to result from the chronic accumulation of
Carolyn V. Nguyen, MD
Sanjay R. Parikh, MD
Children's Hospital at Montefiore
Bronx, NY
Henry M. Adam, MD, Editor, In Brief
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