Canal wall reconstruction and preservation in the surgical management of cholesteatoma in children with Down's syndrome

IntroductionDown's syndrome is associated with poor Eustachian tube function, and an increased incidence of cholesteatoma. The only previously published case series suggests that 'canal wall preserving' procedures are only rarely suitable for the management of cholesteatoma in this population.MethodsWe conducted a retrospective review of the hospital's clinical records database to identify patients with Down's syndrome and cholesteatoma. These patients' notes were then reviewed.ResultsWe identified nine patients with Down's syndrome who had undergone surgical management of cholesteatoma over a twelve year period. Three patients had bilateral disease, meaning twelve ears were treated. Seven ears were initially treated with 'canal wall down' procedures. Four out of five of the remaining ears were successfully treated using 'canal wall preservation' or 'canal wall reconstruction', with one ear requiring subsequent conversion to a 'canal wall down' approach.ConclusionCanal wall preservation/reconstruction is feasible in patients with Down's syndrome, even when cholesteatoma extends into the mastoid.