Congenital Cholesteatoma: Endoscopic Outcomes and Recidivism Risk Factors

Hoff, Stephen R1,2

1 Division of Pediatric Otolaryngology – Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA
2 Department of Otolaryngology – Head and Neck Surgery, Northwestern University, Chicago, IL, USA

Objective: To describe outcomes for the endoscopic resection of congenital cholesteatoma (CC) and risk factors for recidivism

Methods: 30 CCs in 29 pediatric patients (1 bilateral) at a tertiary children’s hospital were followed prospectively from their time of diagnosis to present.  Median age at presentation 4.9 years (range: 14 months – 13.2 years), with a mean follow up of 2.6 years.  Potsic staging: 17% stage I, 17% stage II, 47% stage III, and 20% stage IV.  Transcanal endoscopic resection (EES Type 3) was possible for all Stage I-III patients, and one Stage IV patient (83% total).  The remaining Stage IV patients had all middle ear work done with the endoscopes (EES Type 2b).  The bilateral patient underwent simultaneous resection.

Outcomes tracked include: residual disease, need for second surgery, hearing outcomes, and complications

Results: Early Stage (Potsic I/II), 12 ears:  No ears had residual disease, and none required a second stage.  All had a final ABG < 20 dB.

Advanced Stage (Potsic III/IV), 18 ears: 75% had a planned second stage.  Residual disease was found in 21% of Stage III and 33% of Stage IV ears.  Statistically significant risk factors for residual disease include: advanced stage, “open” CC, stapes erosion, severe inflammation, and higher bleeding score (Modena). Two patients (10%) developed retraction pockets after initial CC surgery, which was repaired at the second stage. These two patients did not have cartilage grafting at first stage.  Overall, 64% of Stage III/IV patients had a final ABG < 20 dB. A majority (5/6, 83%) of patients with Stage IV disease had Medicaid with possible access to care issues.

Conclusion: Endoscopic techniques have favorable outcomes for pediatric CC