Types of Cholesteatoma - Congenital & Acquired Cholesteatoma
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🦻 Types of Cholesteatoma
Cholesteatoma is broadly classified into two major types:
- Congenital cholesteatoma
- Acquired cholesteatoma
- Primary acquired cholesteatoma
- Secondary acquired cholesteatoma
👶 Congenital Cholesteatoma
📖 Definition of Congenital Cholesteatoma
Congenital cholesteatoma is an expanding cystic mass of keratinizing squamous epithelium located medial to an intact tympanic membrane.
Key point: Tympanic membrane is intact
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Assumed to be present at birth
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Usually diagnosed in infancy or early childhood
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No prior history of:
- Otorrhea
- Tympanic membrane perforation
- Ear surgery
🧬 Etiopathogenesis of Congenital Cholesteatoma
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Caused by persistence of embryonic epithelial cell rests
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These rests are located in the epitympanum
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Normally regress during development
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Persistence → accumulation of squamous epithelium in middle ear → cholesteatoma
What are the common sites of Congenital Cholesteatoma?
🩺 Clinical Features of Congenital Cholesteatoma
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Pearly white mass seen medial to an intact tympanic membrane
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Conductive hearing loss
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Often detected:
- Incidentally on routine otoscopy
- During myringotomy
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May rarely rupture spontaneously, presenting as a discharging ear → can mimic CSOM
What is Levenson’s Criteria for diagnosis of Congenital Cholesteatoma?
📊 Potsic Staging System (Congenital Cholesteatoma)
| Stage | Description |
|---|---|
| Stage I | Single quadrant involvement; no ossicular or mastoid involvement |
| Stage II | Multiple quadrants involved; no ossicular or mastoid involvement |
| Stage III | Ossicular involvement without mastoid extension |
| Stage IV | Mastoid extension irrespective of other findings |
🟡 Primary Acquired Cholesteatoma
📖 Why “Primary”?
Called primary because it develops without:
- Prior otitis media
- Tympanic membrane perforation
- Otorrhea
🧠 Theories of Pathogenesis of Primary Acquired Cholesteatoma
- Invagination (retraction pocket) theory
- Basal cell hyperplasia theory
- Metaplasia theory
What are the different theories of Cholesteatoma?
What is Sade's metaplasia theory?
What is Basal cell hyperplasia theory?
What is Retraction Pocket theory?
🔄 Genesis of Primary Acquired Cholesteatoma
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Eustachian tube dysfunction → persistent negative middle ear pressure
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Leads to:
- Pars flaccida / posterosuperior pars tensa retraction pocket
- Keratin accumulation → cholesteatoma
Other mechanisms:
- Repeated subclinical infections → metaplasia of middle ear mucosa
- Basal layer proliferation of epithelium
🔴 Secondary Acquired Cholesteatoma
📖 Definition of Secondary Acquired Cholesteatoma
Occurs in the presence of a pre-existing tympanic membrane perforation, commonly:
- Posterosuperior marginal perforation
- Sometimes large central perforation
🧠 Theories of Pathogenesis of Secondary Acquired Cholesteatoma
- Habermann's Epithelial Migration theory
- Metaplasia theory
What is Habermann's Theory?
🔄 Genesis of Secondary Acquired Cholesteatoma
Two common pathways:
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Repeated infections through perforation → metaplasia of middle ear mucosa → cholesteatoma formation
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Acute necrotizing otitis media → large central or marginal perforation → epithelial migration through perforation → cholesteatoma
📝 Summary Table
| Type | Tympanic Membrane | Key Mechanism |
|---|---|---|
| Congenital | Intact | Persistent embryonic epithelial rests |
| Primary Acquired | Intact | Retraction pocket due to ET dysfunction |
| Secondary Acquired | Perforated | Epithelial migration or metaplasia |
📌 Exam Pearls
- Intact TM + pearly white mass → Congenital cholesteatoma
- Pars flaccida retraction → Primary acquired cholesteatoma
- Marginal perforation + cholesteatoma → Secondary acquired cholesteatoma
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