The Significance of Staging in the Treatment of Congenital Cholesteatoma in Children

Objectives: To analyze the clinical characteristics of congenital cholesteatoma (CC), to explore the risk factors related to recurrence of the disease, and to clarify the importance of staging for treatment. Methods: A total of 87 patients were followed up for more than 5 years, who had undergone surgical procedures for CC from September 2010 to January 2017 in Beijing Children's Hospital, Capital Medical University. Patients with CC were identified in accordance with the following Levenson's criteria. The clinical characteristics of CC on different stage and risk factors related to recurrence of the disease were analyzed. Results: Canal wall up mastoidectomy and tympanoplasty (n = 45), transcanal endoscopic approach (n = 29), and canal wall down mastoidectomy and tympanoplasty (n = 13) was, respectively, performed depending on cholesteatoma extension. Between 2010 and 2013, 20.93% of patients had stage I-II disease, whereas 61.26% had stage I-II disease from 2014 to 2017. Meanwhile, the proportion with stage III-IV disease decreased from 79.07% to 38.64% between these 2 time periods. The preoperative air conduction threshold in patients with stage I, II, III, and IV was, respectively, 23.36 +/- 8.20, 45.40 +/- 12.82, 47.49 +/- 12.03, and 50.37 +/- 11.80 dB. The stage of disease was a significant risk factor regarding recurrence (P = .02). Surgery on patients with stage III-IV disease was performed with the aid of a microscope from 2010 to 2013 and with a microscope and endoscope from 2014 to 2017, which reduced the recurrence rate from 26.92% in the former period to 8.33% in the latter period. Conclusion: Early detection of CC is crucial regarding the facilitation of minimally invasive surgery and reducing complication and recurrence rates. The stage of the disease is a significant risk factor regarding recurrence. The surgery shows us the possibility of reducing the recurrence rate of CC, which is performed under a microscope and an endoscope.