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Congenital Cholesteatomas

Emilia Nymander, MSIV Chicago College of Osteopathic Medicine


Genesys Regional Medical Center Dept. of Otolaryngology & Head and Neck Surgery Student Presentation

Enzymatic keratin debris 4-24% incidence in children 0.12 per 100,000 Male predominance (2-3:1) Effects both ears equally Disease ranges from intratympanic pearls to destruction of middle ear and mastoid
Isaacson, G. "Diagnosis of Pediatric Cholesteatoma." Pediatrics 120.3 (2007): 603-08. Print.

Increased incidence of other congenital malformations

Criteria
1. white mass medial to an intact tympanic membrane

2. normal pars flaccida and pars tensa 3. no previous history of perforation or otorrhea 4. no prior otologic surgery 5. exclusion of canal atresia and intramembranous and giant cholesteatomas 6. Previous otitis media does not exclude congenital cholesteatoma

Causes
Several theories on origin of congenital cholesteatomas No single theory can account for entire clinical spectrum Possible origins of disease middle-ear invagination implantation mucosal metaplasia epidermoid formation

Causes
Embryonic cell rest theory: At weeks 10-33 of development there is epidermoid formation in the anterior epitypanum of the fetal temporal bone epidermoid cells should involute after week 33 Congenital cholesteatoma results from failure of this tissue to involute Only explains how they form in the anterior superior quadrant

Other thoughts:
Invasion theory misdirection of migrating ectodermal cells from the developing external auditory canal into the middle ear Forms the building block for a cholesteatoma Intramembranous Theory [Ruedi] Inflammatory injury to tympanic membrane proliferation of squamous epithelium of the basal layer Formation of protruding cones of epithelium that fuse and can go on to form a cholesteatoma Sade et al. proposed that inflammation of normal middle ear mucosa cause transformation into stratified squamous epithelium Posterior lesions form due to migration of anterior epidermoid tissue posteriorly

Patient Presentation:
Younger patients
2-4 yrs old for anterior superior lesions 12 yrs old for posterior lesions

82% are asymptomatic Acute or chronic OM reported in 40-49% of patients 30-40 dB conductive or sensorineural hearing loss Vertigo, Tinnitus, Facial nerve paralysis (rare) Early disease can be seen on otoscopy

Location
Potsic et al. analyzed data from 172 cases in 167 patients

Potsic, William P. "Congenital Cholesteatoma: 20 Years' Experience at The Children's Hospital of Philadelphia." Otolaryngology-Head and Neck Surgery 126.4 (2002): 409-14. Print.

Location
Posterior superior lesions
occur near the incudostapedial joint Bilateral in 3% of cases Can extend into epitypanum, sinus tympani, or facial recess

Additional Testing
Audiometry, Tympanometry
CT of temporal bone Best radiographic study Often will show a well pneumatized mastoid cavity Hypodense, expansile mass that does not enhance with IV contrast MRI if concerned about intracranial involvement

CT imaging

Isaacson, G. "Diagnosis of Pediatric Cholesteatoma." Pediatrics 120.3 (2007): 603-08. Print.

Richter, Gresham T., and Kenneth H. Lee. "Contemporary Assessment and Management of Congenital Cholesteatoma." Current Opinion in Otolaryngology & Head and Neck Surgery 17.5 (2009): 339-45. Print.

Classification
Open
Disrupted sacs of squamous debris Intact tympanic membrane Increased destructive potential Difficult to differentiate from acquired cholesteatomas

Closed
Keratin filled matrixes Well demarcated margins Higher probability of staying as a single unit

Staging
Stage I
only 1 quadrant involved, no ossicular involvement, no mastoid extension (40%)

Stage II
involvement of multiple quadrants, no ossicular involvement, no mastoid extension (14%)

Stage III
ossicular involvement/erosion but no mastoid extension (23%)

Stage IV
mastoid extension (23%)

Treatment
Surgical removal Type of surgery depends upon:
Extent of disease and structures involved Age at presentation

30% can be removed via a transcanal approach Canal wall up vs. Canal wall down typanomastiodectomy Ossicular reconstruction if needed

Treatment

Richter, Gresham T., and Kenneth H. Lee. "Contemporary Assessment and Management of Congenital Cholesteatoma." Current Opinion in Otolaryngology & Head and Neck Surgery 17.5 (2009): 339-45. Print.

Treatment
Canal wall up
Limited exposure Increased of reoccurrence Easier follow up No mastoid bowl to clean in a small child No water precautions

Canal wall down


Increased visualization Less reoccurrence Periodic cleaning required More difficult to fit hearing aides Water precautions

Prognosis
Reoccur in 30-55% of cases after initial surgical removal Within 8-14 months post operatively if disease limited to middle ear Within 30 months in patients with more extensive disease Patients are followed clinically

Prognosis

Potsic, William P. "Congenital Cholesteatoma: 20 Years' Experience at The Children's Hospital of Philadelphia." Otolaryngology-Head and Neck Surgery 126.4 (2002): 409-14. Print.

Why are these important?


In advanced stage it can Perforate tympanic membrane Predispose to OM, ETD Destroy ossicles Decrease hearing Invade and destroy other structures early diagnosis is important preserve hearing prevent extensive surgeries

Works Referenced
Isaacson, G. "Diagnosis of Pediatric Cholesteatoma." Pediatrics 120.3 (2007): 603-08. Print. Kazahaya, Ken, and William P. Potsic. "Congenital Cholesteatoma." Current Opinion in Otolaryngology & Head and Neck Surgery 12 (2004): 398-403. Print. Kim, S. "Congenital Cholesteatoma of the Tympanic Membrane." Otolaryngology - Head and Neck Surgery 127.4 (2002): 359-60. Print. Lalwani, Anil K. "Congenital Cholesteatoma." Current Diagnosis & Treatment in Otolaryngology - Head & Neck Surgery. Second ed. New York: Lange Medical /McGraw Hill, 2008. Print. Potsic, William P. "Congenital Cholesteatoma: 20 Years' Experience at The Children's Hospital of Philadelphia." Otolaryngology-Head and Neck Surgery 126.4 (2002): 409-14. Print. Richter, Gresham T., and Kenneth H. Lee. "Contemporary Assessment and Management of Congenital Cholesteatoma." Current Opinion in Otolaryngology & Head and Neck Surgery 17.5 (2009): 339-45. Print.

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