gms | German Medical Science

Introduction: A developing external nose deformity severely affects the appearance of granulomatosis with polyangiitis (GPA) patients, often with devastating psychological effects. The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients.

Methods: A systematic literature search was performed for scientific articles archived in the MEDLINE-Database up to June 10^th, 2016, describing management of cases in GPA patients with saddle nose deformity and/or septal perforation.

Results: Eleven of 614 publications met the criteria for this analysis including 41 GPA patients undergoing external nasal reconstruction and/or septal reconstruction with a median follow-up of 2.6 years. Overall, saddle nose reconstruction in GPA patients is safe even if an increased rate of revision surgery has to be expected. Most implanted grafts were autografts of calvarial bone or costal cartilage. For septal perforation reconstruction, few studies were available. Therefore, on current knowledge, surgical outcomes cannot be deduced from the data. All included GPA patients had minimal or no local disease at the time of reconstructive surgery. Therefore, the relationship between disease activity and its impact on surgical outcomes remains unanswered.

Conclusion: This study systematically reviews the efficacy and safety of surgical reconstruction of external nasal deformities in GPA patients for the first time. Saddle nose reconstruction in GPA patients with minimal or no local disease is a safe procedure despite an increased rate of revision surgery. Further research is required regarding the impact of immune-modulating therapy, long-term outcomes and functional outcomes.

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