Objective Patients with congenital external auditory canal (EAC) abnormalities are at risk of developing cholesteatoma and often undergo surveillance imaging to detect it. The aims of this systematic review are to determine the incidence of cholesteatoma in patients with congenital aural atresia (CAA) and patients with congenital EAC stenosis and to investigate the most common age of cholesteatoma diagnosis. This information will help clinicians decide which patients require surveillance scanning, as well as the timing of imaging. Data Sources Ovid MEDLINE, Embase, CENTRAL, and Web of Science databases. Review Methods A systematic literature review following the PRISMA guidelines was performed. The data sources were searched by 2 independent reviewers, and articles were included that reported on CAA or congenital EAC stenosis with a confirmed diagnosis of cholesteatoma. The selected articles were screened separately by 3 reviewers before reaching a consensus on the final articles to include. Data collection on the number of patients with cholesteatoma and the age of diagnosis was performed for these articles. Results Eight articles met the inclusion criteria. The incidence of cholesteatoma was 1.7% (4/238) in CAA and 43.0% (203/473) in congenital EAC stenosis. The majority of patients with congenital EAC stenosis that developed cholesteatoma were diagnosed at age <12 years. Conclusion CAA is associated with a low risk of cholesteatoma formation, and surveillance imaging is unnecessary in asymptomatic patients. EAC stenosis is strongly associated with cholesteatoma, and a surveillance scan for these patients is recommended prior to 12 years of age with close follow-up into adulthood.

Background: Wait time information and compliance with national guidelines are limited to a few adult conditions in the province of Quebec. We aimed to assess compliance with Paediatric Canadian Access Targets for Surgery (P-CATS) guidelines and determine the burden incurred due to waiting for 3 common elective surgical conditions (inguinal hernia, cryptorchidism and hypospadias) in a pediatric population. Methods: We carried out a population-based retrospective cohort study of randomly selected children residing in Quebec without complex chronic medical conditions, using administrative databases belonging to the Régie de l’assurance maladie du Québec for the period 2010–2013. Disability-adjusted life years (DALYs) were calculated to measure the burden due to waiting. Multivariate forward regression identified risk factors for compliance with national guidelines. Results: Surgical wait time information was assessed for 1515 patients, and specialist referral wait time was assessed for 1389 patients. Compliance with P-CATS benchmarks was 76.6% for seeing a specialist and 60.7% for receiving surgery. Regression analysis identified older age (p < 0.0001) and referring physician specialty (p = 0.001) as risk factors affecting specialist referral wait time target compliance, whereas older age (p = 0.040), referring physician specialty (p = 0.043) and surgeon specialty (p = 0.002) were significant determinants in surgical wait time compliance. The total burden accrued due to waiting beyond benchmarks was 35 DALYs. Conclusion: Our results show that provincial compliance rates with wait time benchmarks are still inadequate and need improvement. Patient age and physician specialty were both found to have significant effects on wait time target compliance.

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