Sinonasal Wegener Granulomatosis: A Single-Institution Experience With 120 Cases

Objectives/Hypothesis: Wegener granulomatosis (WG) is intimately associated with the sinonasal tract, with involvement reported in 85% of patients during the course of the disease process. The objectives of the study were: 1) to describe sinonasal symptoms and signs at the time of initial otolaryngologic evaluation; and 2) to review indications and outcomes for rhinologic surgery in WG at our institution. Methods: A retrospective analysis of 120 patients presenting with WG and referred for otolaryngology consultation at a tertiary care center was performed. Results: Eighty-nine percent of patients exhibited sinonasal involvement including nasal crusting (69%), chronic rhinosinusitis (CRS) (61%), nasal obstruction (58%), bloody nasal discharge (52%), septal perforation (33%), saddle-nose deformity (23%), epiphora (13%), and mucocele formation (3.3%). Thirty-nine (33%) patients underwent primary functional endoscopic sinus surgery or dacryocystorhinotomy (DCR) prior to referral. Nineteen (16%) patients underwent noncosmetic, functional sinonasal procedures at our institution. These included endoscopic sinus surgery for CRS or mucocele decompression in 14 patients, DCR for chronic epiphora in seven patients, and orbital decompression for pseudotumor in three patients. Individual symptom recurrence within the first year was greatest for visual impairment from pseudotumor (50%), followed by CRS (14%) and nasolacrimal duct obstruction (11%). Conclusions: The majority of patients with WG are referred to otolaryngology because of involvement of the sinonasal tract. Noncosmetic functional sinonasal procedures are indicated in a minority of patients. Sinonasal morbidity remains significant even after surgery. It is greatest for orbital pseudotumor, but also common for CRS and nasolacrimal duct obstruction.