Cholesteatoma: Is a second stage necessary?

Cholesteatoma can be a difficult disease to treat because the underlying cause of the disease, Eustachian tube dysfunction, is generally not addressed. This can lead to recurrent disease. Surgical resection of cholesteatoma can also be quite challenging, and residual cholesteatoma is often present after surgery. The term recidivism is frequently used to describe both recurrent and residual cholesteatoma. The total recidivism rate has been found in various studies to be between 10% and 70%.1 The primary treatment has been debated for decades, with recommendations for canal wall down (CWD), canal wall up (CWU), staged procedures, and endoscopic procedures. Historically, CWD procedures have lower overall recidivistic rates. Canal wall up with second-look procedures have similar recidivistic rates. Recently, some have advocated foregoing the second look in favor of a planned single-stage procedure with CWD procedures for those with known extensive disease, and CWU procedures for those whom the cholesteatoma can be removed completely.1 The reasoning is that only residual disease is addressed by second-look procedures; if the disease is entirely removed in the primary procedure, second-stage procedures should not be necessary. To minimize any unnecessary procedures, some surgeons recommend two-stage procedures only when cholesteatoma persistence is uncertain. As a response, our group asks this question: “Is a second stage necessary to treat cholesteatoma in canal wall up procedures?” In 2006, Kaylie et al. looked at 470 procedures for cholesteatoma2 and evaluated residual disease and recurrent disease. They performed 183 CWU procedures for cholesteatoma and 287 CWD procedures, with a mean 4.1 years of follow-up. They performed second-stage procedures when complete removal of the cholesteatoma could not be certain. Thirty-six (19.7%) of the CWU procedures were staged. Of those receiving staged procedures, five (13.9%) were found to have residual disease. Residual cholesteatoma was found in 15 (8.2%) patients who did not have planned staged procedures. The overall residual cholesteatoma rate was similar to previous studies, with planned second-stage procedures at 14%. Hanna et al. proposed using a minimally invasive treatment.3 They treated 184 ears for cholesteatoma using either entirely transcanal or CWU mastoidectomy. Follow-up for these patients was between 1 and 11 years, with a mean of 3.2 years. Eighty-three (45%) of these patients underwent planned second-look procedures due to concern for residual cholesteatoma. Seventy-one (39%) of these patients underwent planned second-look procedures without concern for cholesteatoma. Three (2%) of these patients required unplanned second-look procedures for residual cholesteatoma found on follow-up. When combining all of these groups, 15 (8%) of these patients had expected residual disease, and five (3%) had unexpected residual disease during planned or unplanned second-look procedures. Pediatric patients with cholesteatoma have greater variability in presentation because these patients can have congenital or acquired cholesteatoma. McRackan et al. looked at 517 pediatric congenital and acquired cholesteatomas.4 They performed second-look procedures in patients for whom there was a concern for residual cholesteatoma at the time of surgery. There were 244 (47.2%) planned second-look procedures, of which 117 (48%) had residual cholesteatoma. Of the 273 patients in their study who did not have second-look procedures, 13 (4.4%) had residual or recurrent cholesteatoma, demonstrating low residual rates in those patients who were considered to have complete removal. Other techniques have become recognized in treating cholesteatoma, including the use of endoscopes during cholesteatoma surgery. Neudert et al. evaluated 406 procedures comparing tympanoplasty with endoscopic evaluation (45 procedures), CWU mastoidectomy (321 procedures), and CWD mastoidectomy with or without obliteration of the mastoid bowl (40 procedures).1 They used an algorithm to determine the extent of the surgery. Patients in whom the canal was intact preoperatively underwent tympanoplasty with endoscopic evaluation or canal wall up tympanomastoidectomy. There were 44 planned second-look procedures, and nine (20.45%) residual cholesteatomas were found. When the authors evaluated all residual tumors, both from planned second-look and unexpected residual tumors, they found residual tumors in 6.5% of all CWU procedures and in 7% of all CWD procedures. This represents 4.5% of unexpected residual cholesteatoma in CWU procedures. Previously, computed tomography scans have been trialed to evaluate for recurrent/residual cholesteatoma, with poor results due to low specificity.5 Jindal et al. performed a systematic review of diffusion- weighted imaging, evaluating both echo planar imaging (EPI) and nonecho planar imaging (non-EPI) techniques.5 Sixteen studies were included with a total of 432 patients. Echo planar imaging had a sensitivity of 70.6%, specificity of 87.3%, positive predictive value (PPV) of 91.4%, and negative predictive value (NPV) of 74.1%. Non-EPI had improved results with sensitivity of 91.4%, specificity of 95.8%, PPV of 97.3%, and NPV of 85.2%. The authors found that cholesteatoma as small as 2 mm could be predictably found, but there were a few limitations of this study. First, not all findings were confirmed with second-look procedures. Second, none of the studies were randomized controlled studies. Second-look procedures keeps overall recurrence from residual cholesteatoma rates similar to that of CWD procedures.1 In the four studies presented here, 1,250 patients were treated with CWU procedures and 49 (3.9%) unexpected residual cholesteatomas were found. 1-4 Four hundred seventy-eight planned second-look procedures were performed and 156 (32.6%) residual cholesteatomas were found. Therefore, more than 67% of the second-look procedures were unnecessary. Finally, new methods, such as nonecho planar imaging magnetic resonance imaging (MRI) for evaluation of postoperative ears, offer highly sensitive and specific evaluation of recurrent and residual cholesteatoma. Use of this imaging modality may help prevent unnecessary surgery for patients when there is concern for residual or recurrent cholesteatoma.5 Recently, many have favored a tailored approach, with a second look reserved for those patients when cholesteatoma was thought to be left behind at the primary surgery.. The studies presented here show that a selective approach to staging cholesteatoma surgery, in both adults and pediatric patients, is effective and appropriate and will reduce unnecessary procedures.4 Second-look procedures should be used for patients when complete removal of the disease is uncertain. Nonecho planar MRI evaluation may be used for patients who have concerned but uncertain findings on postoperative physical exam. Mandatory second-look procedures for CWU surgery are not necessary. Currently there is no level 1 evidence available. The articles in this review show level 2b evidence.