Residual cholesteatoma of the temporal bone in a patient with granulomatosis with polyangiitis

Abstract

A case of residual temporal bone cholesteatoma in a patient with granulomatosis with polyangiitis (Wegener) (GPA) is presented. At the onset of the disease, the patient developed a symptom complex, which included, along with sinonasal manifestations, left-sided purulent otitis media, refractory to traditional therapy. A separate atticoanthrotomy on the left temporal bone, performed in the active phase, before the diagnosis of vasculitis, did not lead to a clinical improvement in the condition of the middle ear. Despite the subsequent implementation of adequate immunosuppressive therapy and drug remission of GPA, the continuously recurrent course of purulent otitis media of the operated ear contributed to the development of residual cholesteatoma of the temporal bone with the spread of the process to surrounding structures. The presence of cholesteatoma was confirmed by MRI of the temporal bones in the non-EPI DWI mode. During the period of drug remission of GPA, repeated sanitizing surgery was performed on the middle ear with tympanoplasty and obliteration of the parathympanic spaces, which made it possible to achieve remission of purulent otitis media. The development of residual middle ear cholesteatoma in a patient with GPA could be due to the untimely initial diagnosis, the late onset of specific immunotherapy, the rapidly progressive and recurrent nature of the course of the disease, and surgical treatment in the phase of high disease activity. The insufficient volume of primary surgical treatment contributed to the formation of laced cavities and the development of residual cholesteatoma of the temporal bone. The use of MRI in the non-EPI DWI mode for diagnosis made it possible to identify the development of cholesteatoma of the left temporal bone in a patient with GPA and track the results of surgical treatment in the postoperative period. The non-EPI DWI MRI method proved effective both for detecting temporal bone cholesteatoma and tracking changes in the postoperative period of a patient with GPA. Tympanoplasty with surgical obliteration of the parathympanic spaces allowed to stop the inflammation of the middle ear in a patient with a systemic autoimmune disease.