Comparison of current staging systems for predicting pediatric cholesteatoma outcomes

This study aims to assess the utility of the European Academy of Otology & Neurotology - Japanese Otologic Society (EAONO-JOS) and Potsic staging systems in predicting recidivism in pediatric patients with congenital (CC), primary acquired (PA), and secondary acquired (SA) cholesteatoma.

This is a retrospective study on 31 ears from 30 pediatric patients (≤18 years old) treated from 2015 to 2023 for CC, PA, and SA cholesteatoma. Surgical ears were classified according to EAONO-JOS and Potsic staging system. Surgery included transcanal endoscopic ear surgery (TEES), canal-wall up (CWU) or canal-wall down (CWD) mastoidectomy. Primary outcomes included the rate of residual disease at second-look surgery and the rate of recurrence at clinical observation. In addition, stapes erosion, incus erosion, labyrinthine fistula, and extension of disease in the mastoid at primary surgery were investigated for predictivity for recidivism. Descriptive statistics, Kaplan-Meier estimators (KM), and Fisher's Exact tests were used for statistical analysis.

Based on EAONO-JOS staging, the majority (87 %) of cholesteatoma were stage II (100 % for CC, 86.7 % for PA, and 60 % for SA). The rate of residual disease was 45 % for CC, 60 % for PA, and 40 % for SA cholesteatoma. The rate of recurrent disease among the entire cohort was 6.5 %. Univariate analysis on stapes or incus erosion or mastoid extension did not predict residual disease. Within the CC cohort, outcomes suggest a potential correlation between Potsic stage and the risk of residual disease. Insufficient variability in EAONO-JOS stages precluded statistical analysis of the system's ability to predict residual disease. Among the EAONO-JOS stage II cases, those presenting with all three variables (mastoid extension, incus erosion, and stapes erosion) at primary surgery had lower KM survivability (p = 0.010). The type of surgery was predictive of residual disease: CWU was associated with a lower KM survivability compared to TEES (p = .009). CWD was not predictive, given the insufficient sample size. Among all ears, 58 % were managed with TEES.

In this limited cohort of pediatric CC, PA, and SA cholesteatoma, the majority of cases were managed with TEES. Among EAONO-JOS stage II cases, the simultaneous presence of mastoid extension, incus erosion, and stapes erosion at primary surgery demonstrated statistically significant decreased residual-free survivability; TEES approach at primary surgery also has a statistically significant improved residual-free survivability compared to CWU approach.