Congenital Cholesteatoma: Endoscopic Outcomes and Recidivism Risk Factors

By June 7, 2019

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.

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

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

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.

Endoscopic techniques have favorable outcomes for pediatric CC.