Long-Term Follow-up of Repair of External Nasal Deformities in Patients With Wegener's Granulomatosis

David Congdon, MD, MPH; David A. Sherris, MD; Ulrich Specks, MD; Thomas McDonald, MD

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. Study Design: 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. Key Words: Wegener's granulomatosis, saddle nose, nasal deformity, septorhinoplasty.

INTRODUCTION

Wegener's granulomatosis (WG) is a vasculitic disease involving the upper airway, lungs, and kidneys. The disorder was first described by Heintz Klinger' in 1931 as a borderline form of polyarteritis nodosa. In 1939, Friedrich Wegener published a detailed study of this new disease, "A Peculiar Rhinogenic Granuloma With Particular Involvement of the Arterial System and Kidney," distinguishing it from polyarteritis nodosa.

Fig. 1. Graft material used for dorsal defects.

Fig. 2. (A) The septal cartilage has totally dissolved between the medial crural feet. (B) The split calvarial bone graft is in place, and multiple 6-0 nylon interdomal sutures fixate the lower lateral cartilage together. (C) Frontal view before rhinoplasty. (D) Frontal view after rhinoplasty.

Wegener's granulomatosis affects three main areas in the body as demonstrated by the "ELK" eponym. The "E" represents ENT for head and neck involvement, "L" represents lungs for pulmonary involvement, and "K" rep-resents kidney f'or the renal involvement." The disease can present with any combination of these locations affected, although the diagnosis is most obvious when all three sites are involved. When the head and neck area is in­volved in isolation, the diagnosis becomes more elusive. Other diagnostic modalities must be used to secure a correct diagnosis in these cases. These include the c-ANCA test and tissue biopsy.

Before 1985, a specific laboratory test for WG did not exist. The c-ANCA blood test available today is highly specific for WG." A negative c-ANCA test result does not exclude the diagnosis of WG. The sensitivity of the test is limited and depends on the extent and activity of the disease at the time the study is performed. Its specificity is greater than 90% during the systemic vasculitic phase of the disease but only approximately 65% in patients with predominantly granulomatous disease manifestations limited to the upper or lower respiratory tract, or both. In patients with no signs of disease activity (complete remis­sion), the sensitivity is approximately 30%.''

Fig. 2. (Continued) (E) Oblique view before rhinoplasty. (F) Oblique view after rhinoplasty.

Histopathological confirmation of clinical impres­sions is important in all cases. The histological diagnosis of WG is based on three microscopic findings: parenchy­mal necrosis, vasculitis, and granulomatous inflamma­tion.' Nasal biopsy is typically the simplest to obtain. Multiple, deep biopsy specimens of the septum and intra­nasal mucosa are necessary to identify these key histolog­ical findings. Once diagnosed, the treatment of WG with agents including cyclophosphamide, prednisone, metho­trexate, and trimethoprim sulfamethoxazole is left up to the pulmonologist, immunologist, nephrologist, or other local expert. Up to 75% of patients treated with steroids and cyclophosphamide reach complete remission.

The nasal manifestations of the WG commonly results in destruction of the cartilaginous support of the nose and a severe saddle-nose deformity. The devastating psychological effects of a saddle nose in the chronically ill patients with WG is especially damaging. In WG the cartilaginous struc­tures of the nose and septum are usually more severely involved and eroded than the bony nasal dorsum and sep­tum, although both cartilage and bone can be affected simul­taneously by the granulomatous vasculitis.

On review of the literature, no reports were located discussing reconstruction of this devastating disorder in the WG patient. Because the recipient site for the graft (nasal dorsum) is abnormal, some authorities hypothe­size that reconstruction would be fraught with higher complication rates, failure rates, graft resorption rates, and so on. Many physicians may even refuse to perform reconstruction in these patients because of the WG and chronic medications that have an adverse effect on wound healing (e.g., prednisone, cyclophosphamide). For this reason, we thought it important to review our experience with external nasal reconstruction in this unique group of patients.

PATIENTS AND METHODS

A retrospective chart review was undertaken of all of the patients with WG who underwent external nasal deformity re-construction at the Mayo Clinic between 1976 and 2000. Patients who underwent septoplasty or repair of septal perforation were excluded.

Fig. 2. (Continued) (G) Left lateral view before rhinoplasty. (H) Left lateral view after rhinoplasty. (I) Skyline view before rhinoplasty. (J) Skyline view after rhinoplasty.

The criteria for diagnosis of WG were history, physical ex­amination, biopsy findings consistent with WG, and laboratory testing including c-ANCA when available. If there was uncer­tainty about the diagnosis, the patient was removed from the study group.

Notes from the otolaryngologist who performed the surgery and the other specialties involved in the medical aspects of the WG were reviewed. Photographs before and after surgery were reviewed when available.

RESULTS

Thirteen patients qualified for the study. The aver-age patient age was 43 years (range, 24-73 y). Sixty-two percent of patients included in the study had a biopsy finding that was consistent with WG. Of patients who had the c-ANCA test available, 67% were c-ANCA positive. Four patients were treated before the availability of the c-ANCA test. In patients for whom biopsy was not diag­nostic, the diagnosis was based on clinical grounds or c-ANCA testing, or both.

Thirteen patients underwent a total of 16 surgical procedures. The average duration of follow-up was 59 months with a median follow-up of 33 months and a range of 10 to 177 months. One patient had a graft infection and was lost to follow-up after its removal at 2 months. Female patients predominated, accounting for 12 of 13 patients.

The repairs included 12 dorsal augmentations for saddle-nose deformity, 1 composite graft to repair a defect of the nasal ala, and 3 revision dorsal augmentation pro­cedures, for a total of 16 surgical procedures. The most common graft materials used in the reconstruction of the dorsal deformities included 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) (Fig. 1). The single alar rim defect was repaired using a composite auricular graft.

Success and Failure Related to First Reconstruction

An outcome was considered favorable and successful when the patient was satisfied with the appearance of the nose. The initial success rate was 77% with 10 of 13 reconstructions resulting in a favorable outcome with a single surgery. Of the 12 dorsal augmentations, 75% (9 of the 12 repairs) resulted in a successful outcome with the primary surgery (Table I). The single composite recon­struction of the nasal ala was a success.

The patient shown in Figure 2A-J is a typical exam­ple of a patient with WG and an associated saddle-nose deformity. She had reconstruction using a cantilevered split calvarial bone graft dorsal augmentation. This pa­tient had a satisfactory result after the primary surgery and had no associated local or systemic flare-up of WG.

There were three cases that resulted in treatment failure following the primary surgery. Two of the primary surgery failures were secondary to resorption of the graft, and the third failed secondary to postoperative wound infection. The two patients with graft resorption under-went revision surgery, and the patient with wound infec­tion declined further surgery.

The two primary graft resorption failures occurred at 10 and 26 months postoperatively, and the wound infection occurred in the immediate postoperative period. The infected graft was removed on postoperative day 46. One of the revi­sion surgeries was a success; the other failed secondary to graft resorption 8 months after surgery (Fig. 3). The revision failure was able to be salvaged with a second revision, which was successful. The overall success rate was 92% with 12 of 13 patients achieving a satisfactory final result (Table I).

Ten of the 13 patients who underwent reconstruction had an improvement in their nasal airway. Seven were described as having significant improvement, and three were described as having some improvement postopera­tively. The three remaining patients had no mention of the postoperative airway in the medical record. In none of the patients was the nasal airway made worse as a result of the reconstruction (Fig. 4). The patients with graft resorp­tion who underwent revision surgery eventually achieved an improved nasal airway. None of the patients in the study had mention of a worsening of the nasal airway postoperatively.

Complications

Beyond the single early wound infection, there were no cases of epistaxis, hematoma, wound dehiscence, or other early infections. The overall rate of early complica­tions was 6% (1 of 16).

TABLE I.

Outcome of Primary Reconstruction.

Type of Reconstruction	No. of Patients	Percent Successful (no.)
Dorsal deformity	12	75%	(9/12)
Nasal ala deformity	1	100%	(1/1)
Total	13	77%	(10/13)

Outcome of Revision Surgery

Revision Number	No. of Patients	Percent Successful (no.)
First revision	2	50%	(1/2)
Second revision	1	100%	(1/1)
Total patients	2	100%	(2/2)
undergoing revision

The late complications of graft resorption occurred 3 times in 16 operations (19%). The materials that resorbed were irradiated rib (1 of 1), irradiated dura mater (1 of 1), and calvarial bone (1 of 4). The success rates for calvarial bone and costal cartilage were 75% and 83%, respectively. The two cases that used irradiated materials (rib and dura) failed (Table II). There were no cases of local or systemic flare-up of WG and no cases in which the disease accelerated in the postoperative period. We did not see any cases in which skin envelope contracture was a problem in the current series.

TABLE II. Success Rates for Individual Graft Types
Type of Graft Material	No. of Grafts	No. of Failures	Percent Successful (no.)
Costal cartilage	6	1	83%	(5/6)
Calvarial bone	4	1	75%	(3/4)
Composite auricular	1	0	100%	(1/1)
Iliac bone	1	0	100%	(1/1)
Conchal cartilage	1	0	100%	(1/1)
Septal bone	1	0	100%	(1/1)
Irradiated rib	1	1	0%	(0/1)
Irradiated dura	1	1	0%	(0/1)
Total grafts	16

The first graft resorption occurred approximately 2 years after the original reconstruction and resulted in a return of the saddle-nose deformity. The original repair was performed using irradiated rib, and the secondary augmentation used irradiated dura. This improved the appearance for 8 months, when augmentation of the dor­sum had to be repeated with calvarial bone. This final revision was successful with 1-year follow-up.

The second graft resorption case occurred 10 months after the original reconstruction and consisted of partial supratip collapse of the calvarial bone graft. This was repaired using auricular cartilage and irradiated dura as an onlay graft. This revision corrected the deformity, and the patient was happy with the results 4 years after the original surgery.

The final case of failed nasal reconstruction involved a postoperative wound infection of a costal cartilage graft. The patient failed intravenous antibiotic treatment of the infection, and the graft had to be removed 46 days after the original surgery. The patient recovered quickly after removal of the graft and did not seek a revision operation. Wegener's granulomatosis disease severity was examined as it related to the success of the nasal reconstruction. Of the total of 13 patients, 8 had only upper airway involvement, or "E" WG by the ELK eponym (see "Introduction"), at the time of the operation. The remaining five patients had both upper airway involvement and pulmo­nary involvement or "EL" WG. None of the patients in our series had any renal manifestation of WG. Of the patients with limited or "E" WG, the success rate was 88% (seven of eight primary reconstructions). Of the patients with the more severe "EL" WG, the success rate was 60% (three of five primary reconstructions) (Fig. 5). The single failure in the "E" WG group was the patient who had the greatest resorption of the irradiated rib graft and required two repeat operations. The two "EL" WG failures consisted of the wound infection and the mild supratip resorption of the calvarial bone graft.

DISCUSSION

One of the more devastating psychological effects of WG is the external nasal deformity that results from destruction of the cartilaginous framework of the nose. By far the most common external nasal deformity seen with WG is the dorsal deformity. Before the current review, no study has addressed the long-term sequelae of recon­structing the nasal deformity associated with WG. The hesitation by some physicians to reconstruct these defects stems from concerns that an attempt to repair the nose would result in a flare-up of the WG both locally and systemically.

No perioperative flare-ups of WG either locally or systemically occurred in the current group of patients. The failures that did occur were independent of any reactiva­tion of the disease. The question of whether the patient has to be in remission with minimal or no local disease remains unanswered because all of the patients who had surgery had mild or no symptoms at the time of the re-construction. This is because of the institutional philoso­phy that it is best to have the patient in remission at the time of the repair.

Disease severity did seem to affect the chance for a successful outcome, but because of the small number of cases in the current study, no definitive conclusions can be made. The success rate was only 60% for the "EL" WG group compared with 88%, for the "E" WG group. The failure case in the "E" WG group that required two revi­sions was probably related to the use of irradiated mate­rials for reconstruction. Because of this, it appears that one should avoid using irradiated homografts, since two of two irradiated grafts failed. Autogenous materials, such as costal cartilage and calvarial bone, show great promise in these patients.

The two failures in patients who underwent a revi­sion surgery eventually had a favorable outcome and were happy with the final result. Neither patient had any ad-verse effects secondary to either the primary or secondary reconstruction. Taking this into account, nearly every pa­tient undergoing reconstruction was able to achieve a desirable final result with no major adverse sequelae.

The revision rate for patients with WG undergoing external nasal reconstruction appears to be higher than in the typical septorhinoplasty population, and preoperative counseling about this fact is warranted. The published success rate for dorsal augmentation varies by study but has usually been approximately 95% for the primary sur­gery.' Our success rate of 77% for the primary reconstruc­tion compares less favorably in this patient population.

CONCLUSION

The reconstruction of nasal deformities in patients with WG is a safe and effective procedure. Patients should be in remission before any reconstructive effort is under-taken, to minimize complications related to both high-dose systemic steroids and theoretical concerns of active dis­ease response.

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