LANDMARK: Children’s Hospital of Philadelphia (CHOP) Congenital Cholesteatoma Experience

Article: Congenital cholesteatoma: 20 years’ experience at The Children’s Hospital of Philadelphia.

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

Take Home Points:

  • Previously thought that congenital cholesteatomas originated from embryonic cell rest in anterior superior quadrant, however, there was a subset of posterosuperior quadrant in this study conflicting the embryonic cell rest theory. 
  • Location of cholesteatoma is related to patient’s age with anterosuperior disease more common at younger age
  • Location of disease relates to post-op hearing results and likelihood of residual or recurrent disease. Mastoid and ossicular chain involvement needs closer observation.

The Details:

  • Retrospective chart review of all patients seen at CHOP who underwent surgery for cholesteatoma between July 1, 1981, and December 31, 2000. 
  • Congenital origin for cholesteatoma was defined as an intact tympanic membrane with normal pars tensa and pars flaccida w/o history of previous perforation or otorrhea and no history of previous otologic surgery. 
  • Patients with a history of acute otitis media w/o perforation or serous otitis media were not excluded
  • 172 cases in 167 patients met inclusion criteria for congenital cholesteatoma
    • Average age of patient at time of surgery- 5.0 years, average follow-up 2.8 years
    • High preponderance for boys (72%), no predilection for side: Right (46%), Left (54%).
    • Most common presentation was asymptomatic middle ear mass (82%), 13% found at myringotomy for serous otitis media
    • 40% prior history of at least 1 episode of of acute otitis media, 21% completely negative otologic history
  • Most commonly affected site: 81% at anterior superior quadrant of middle ear, 49% at posterior superior quadrant, 42% had ossicular chain involvement. 23% had mastoid extension
  • Single quadrant (anterior superior) most likely to occur in younger ages (<2-3)
  • Staging as presented at 16th annual American Society of Pediatric Otolaryngology
    • 40% Stage I (involving only 1 quadrant with no ossicular involvement nor mastoid extension)
    • 14% Stage II (involvement of multiple quadrant but not ossicles or mastoid) 
    • 23% Stage III (ossicular erosion but not mastoid involvement)
    • 23% Stage IV (mastoid involvement)
  • 111 patients who were old enough to undergo hearing test for preop speech reception threshold. Threshold directly related to stage of disease. 
    • 11 dB with single quadrant Stage 1 progressing to 33 db in stage IV
    • See fig 4 for complete breakdown of speech thresholds
  • Presence of ossicular or mastoid involvement associated with high rates of residual disease: 41% and 67% respectively. 

Contributed by Sahil Patel