Learning Objectives: To enhance understanding of the factors which contribute to development of recurrent cholesteatoma in children as part of the Round Table session on Paediatric Cholesteatoma.
Predictive factors for recurrent cholesteatoma
Recurrent cholesteatoma (i.e. development of new disease after previous surgical clearance) may be a consequence of persistent pathogenetic factors and perhaps also of surgical technique. Cholesteatoma is commonly considered to behave more aggressively in children than adults. A clear understanding of factors that predispose to recurrent disease in children may help selection of optimal surgical technique for disease control for example between canal wall up (CWU) or down (CWD) and mastoid preservation or obliteration.
In an attempt to clarify this relationship between disease, patient and surgery, a single-surgeon prospective database of consecutive cholesteatoma surgeries was analysed. Analysis was restricted to cases with no prior history of cholesteatoma surgery. Out of 368 paediatric cases, 328 (89%) were completed with CWU of which 69 were totally endoscopic (TEES), and 40 were completed with CWD of which 10 had primary obliteration. Overall 34 (9%) were found to develop recurrent cholesteatoma. Kaplan Meier survival (KM) analysis was used to control for the cumulative increase in recurrence with time, giving an overall recurrence rate of 12% at 5 years and 18% at 10 years. Perhaps surprisingly, KM analysis suggests that gender and younger age had no impact on likelihood of recurrence. Similarly the same rate of recurrence was found for congenital and acquired cholesteatoma and whether disease was acquired from pars tensa or pars flaccida retraction. The most significant pre-operative determinant of outcome was extent of cholesteatoma, with cholesteatoma involving 4 sub-sites (meso- and epitympanum, antrum and mastoid) having significantly greater risk of recurrence than smaller disease (30% at 5 years; KM log rank statistic p = 0.002).
While canal wall down surgery (CWD) is commonly considered to have a lower risk of recurrence than intact canal wall surgery (CWU), in this series, KM analysis showed no difference in rate of recurrent cholesteatoma between these different techniques. Subgroup analysis, in which the sample sizes are small, suggests (a) the same recurrence rate with TEES and (b) no difference in revision surgery for uncontrolled disease for CWD cases with or without obliteration of the mastoid. In contrast to the perhaps unreachable gold-standard of a randomized prospective trial to control for differences between patients, it must be noted that selection of surgical technique was allocated according to patient and disease factors, for example CWD surgery was used more often for larger cholesteatoma.
Careful recording and analysis of surgical intervention and outcome provides valuable insight into the effectiveness of otologic intervention for cholesteatoma.
Survival analysis is required to control for the increasing incidence with longer follow up. It is also important to control for other risk factors such as the extent of cholesteatoma. Understanding of the mechanisms of recurrent cholesteatoma is confounded by selection of surgical technique according to the extent of disease.
Nevertheless, as it seems that “Bad ears do badly and good ears do well” it is arguably most important maximise strategies to prevent recurrent disease in those thought to be most at risk.