title: Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis.
abstract: OBJECTIVE
  Wegener's granulomatosis is a disorder of the upper airway, lungs, and kidneys characterized histologically by necrotizing granulomas and vasculitis. One common result of Wegener's granulomatosis is destruction of the framework of the nose, resulting in a saddle-nose deformity. The study was initiated to determine the efficacy and safety of reconstructing external nasal deformities in these patients.
  
  
  METHODS
  A retrospective review.
  
  
  METHODS
  We searched for all patients with Wegener's granulomatosis who underwent an external nasal reconstruction between the years of 1976 and 2000.
  
  
  RESULTS
  Thirteen patients met the search criteria. The average duration of follow-up is 59 months (range, 10-177 mo). The initial surgery resulted in a satisfactory outcome in 10 of 13 patients (77%). Two of the patients who failed the primary surgery underwent successful revision rhinoplasty, and one chose not to undergo revision surgery. This resulted in a 92% (12 of 13) overall patient success rate. Ten of the 13 patients had an improvement in their nasal airway postoperatively, and the remaining three had no mention of postoperative airway in the record. The postoperative airway was not made worse in any of the patients operated on in the series. All of the patients had reconstruction when their Wegener's granulomatosis was in remission. Twelve of the 13 patients with an external deformity had a saddle-nose defect. The remaining deformity was a defect of the alar rim. Sixteen surgeries were preformed in total (15 dorsal repairs [12 primary, 3 revisions] and 1 alar rim repair). The most common graft materials used in the reconstruction of the dorsal deformities were costal cartilage (40% [6 of 15]) and calvarial bone (27% [4 of 15]). The remaining dorsal defects were repaired with irradiated rib (1 of 15), irradiated dura (1 of 15), conchal cartilage (1 of 15), iliac crest (1 of 15), and bony septum (1 of 15). The alar rim defect was repaired using a composite auricular graft. The overall success rates by graft type were as follows: costal cartilage, 83% (5 of 6); calvarial bone, 75% (3 of 4); composite auricular, 100% (1 of 1); iliac bone, 100% (1 of 1); conchal cartilage, 100% (1 of 1); septal bone, 100% (1 of 1); irradiated rib, 0% (0 of 1); and irradiated dura 0% (0 of 1).
  
  
  CONCLUSIONS
  Reconstruction of external nasal deformities in patients with Wegener's granulomatosis is safe. Surgery does not appear to either induce a flare-up or accelerate the course of Wegener's granulomatosis. There may be a higher risk of failure in primary nasal dorsal repair in patients with Wegener's granulomatosis than in other patients undergoing dorsal augmentation. Irradiated materials in the series also appear to be more prone to resorption.
authors.affiliation: Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota 55905, USA.
authors.forename: David
authors.initials: D
authors.lastname: Congdon
authors.forename: David A
authors.initials: DA
authors.lastname: Sherris
authors.forename: Ulrich
authors.initials: U
authors.lastname: Specks
authors.forename: Thomas
authors.initials: T
authors.lastname: McDonald
mesh_terms: D000328:Adult; D016025:Bone Transplantation; D002356:Cartilage / Q000637:transplantation; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006801:Humans; D008297:Male; D009300:Nasal Septum / Q000601:surgery; D009667:Nose Deformities, Acquired / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D012225:Rhinoplasty; D013997:Time Factors; D016896:Treatment Outcome