ORIGINAL ARTICLE

Reconstruction of Nasal Deformity in Wegener’s Granulomatosis:Contraindication or Benefit?

Peter M. Vogt • Andreas Gohritz • Marion Haubitz •

Andreas Steiert

Received: 14 April 2010 / Accepted: 22 July 2010

� Springer Science+Business Media, LLC and International Society of Aesthetic Plastic Surgery 2010

Abstract

Background Saddle-nose deformity is a well-recognized

stigma of patients affected by Wegener granulomatosis

(WG). However, plastic surgical repair is seldom per-

formed. In this study, the authors aimed to evaluate their

own patients exclusively reconstructed by costal cartilage

L-strut of the nose for this specific deformity.

Methods During a 5-year-period, four women with an

average age of 33 years underwent reconstructive rhino-

plasty of their saddle-nose deformity caused by WG, which

in every case was in remission regarding the nose at the

time of surgery. Restoration of the nasal framework was

performed by an L-shaped rib cartilage graft.

Results The external form and function of the newly

reconstructed nose was preserved during an average

follow-up period of 42 months for all the patients. No

resorption of the rib cartilage graft was observed. A review

of the literature found a total of 22 nasal reconstructions for

patients affected by WG.

Conclusion According to this patient series and a review

of the literature, external nasal reconstruction for patients

affected by WG appears to be safe and effective if the

disease is in remission before any operation. Despite con-

cern that high-dose immune suppression therapy may

increase the risk of failure in primary nasal dorsal repair,

this could not be observed in the patients of this series, all

of whom were receiving immunosuppressive medication.

Therefore, nasal reconstruction to improve the physical

appearance and thus the psychological well-being of these

chronically ill patients seems to be justified.

Keywords Costal cartilage � Immunosuppression �Long-term results � L-strut � Saddle-nose deformity �Wegener’s granulomatosis

Wegener’s granulomatosis (WG) is an idiopathic necro-

tizing vasculitis affecting primarily the upper respiratory,

pulmonary, and renal systems. Wegener [16] published his

first detailed report of this disorder in 1939. Formerly, if

left untreated, the disease was a lethal within about

6 months, primarily due to renal and lung involvement.

Currently, up to 90% of patients with WG may reach

complete remission using modern medication [8]. Never-

theless, as many as 23% of WG patients will continue to

suffer from a highly disfiguring nasal deformity [2] because

the nose and sinuses are the first disease manifestation in

most of the patients, who are prone to smouldering or

relapsing disease with destruction of the small vessels by

granulomatous and necrotizing nongranulomatous vascu-

litis. Nasal crusting is associated with foul-smelling, fria-

ble, ischemic, or ulcerated tissues, ultimately leading to

septal perforations and loss of the cartilage support of the

nose with consequent saddle-nose deformity and a short-

ened nose [1, 5].

Reconstruction of these defects usually requires

replacement of dorsal and columellar support and may be

limited by deficits in the nasal lining [11, 12]. Although

saddle-nose deformity is recognized as a classical feature

of this disease, surgical reconstruction often is not even

P. M. Vogt � A. Gohritz � A. Steiert (&)

Department of Plastic, Hand and Reconstructive Surgery,

Hannover Medical School, Carl-Neuberg-Strasse 1,

30625 Hannover, Germany

e-mail: steiert.andreas@mh-hannover.de

M. Haubitz

Departement of Nephrology, Hannover Medical School,

Carl-Neuberg-Strasse 1, 30625 Hannover, Germany

123

Aesth Plast Surg

DOI 10.1007/s00266-010-9568-9

mentioned [2, 3]. Only a few reports have addressed the

potential benefit of nasal reconstruction for patients affec-

ted with this psychologically devastating secondary result

of WG.

As the recipient site for the graft, the nasal dorsum may

be altered by chronic inflammation, higher rates of com-

plications, graft failure, and resorption. Other complica-

tions have been hypothesized. Nasal reconstruction in WG

may even have been rejected due to the relapsing course of

the disease and the chronic medications that have adverse

effects on wound healing (e.g., prednisone, cyclophos-

phamide, methotrexate) [4].

Patients and Methods

A retrospective chart review included four women in our

department between 2001 and 2006 who had reconstructive

rhinoplasty due to severe-saddle nose deformity caused by

WG. All four patients had severe destruction of their

external nasal cartilage frame with subsequent saddle-nose

deformity. The diagnosis was determined according to

the Chapel-Hill consensus criteria and the ACR criteria

[10, 17, 18]. All four women had a positive antineutrophil

cytoplasmic antibody (c-ANCA).

Pre- and postoperative photographs were reviewed.

A period of 18 months was defined as the minimum

follow-up period. Patients who underwent only repair of

septal perforation were excluded from the study.

Operative Technique

For L-strut reconstruction, an open rhinoplasty approach

was chosen. Using a step incision of the columella, the

dorsal skin was elevated and a space dissected to fit the

long leg of the cartilage strut. In case of a residual carti-

laginous septum, the septum was dissected to obtain local

support for the distal part of the L-strut. Then the desired

length and height of the strut was defined. A slightly curved

incision in the submammary fold provided access to the

eighth or ninth rib cartilage.

After incision of the fascia and splitting of the overlying

rectus and serratus anterior muscle, the desired size and

shape of the L-strut was outlined with a surgical marker,

after which the cartilage was incised with a no. 11 blade.

The graft then was excised with the knife in its gross

dimensions. The donor site was closed in layers, with

careful approximation of the tissue to avoid obvious scar-

ring. The strut then was carved in multiple steps to generate

a perfect fit in the position of the roof of the nose, espe-

cially in the portion of the former distal septum. The

medial crura of the alar cartilage was fixed to the short leg

of the L-strut using transparent 4-0 nylon U-sutures. If

needed, an alar dome or spanning sutures were applied to

improve the projection of the nasal tip. The distal part of

the short leg of the L-strut was inserted into a pocket

created close to the bony nasal spine and fixed with another

4-0 suture guided through a drill hole in the nasal spine.

Next, the skin was redraped, and the cartilage was

controlled for irregularities. If necessary, additional trim-

ming was performed. The strut chosen was as large as

necessary for optimal elevation of nose projection in height

and length whereby the possibility for closing the colu-

mella skin incision was the limit of the graft size.

After closure of the nasal skin at the columella with 6-0

nylon and the mucosa with 5-0 Vicryl, skin tapes, a plaster,

and a nasal splint were applied. The nylon stitches were

removed on day 6, and the inner splint was removed in 2 to

6 days. The plaster was removed after 14 days.

Results

Between 2001 and 2006, four women with an average age

of 33 years (range, 26–42 years) underwent surgical cor-

rection of their nasal deformity caused by WG. At the time

of surgery, the disease was in remission for all the patients

except one, who still had a hydromyrinx.

The median follow-up period was 42 months (range,

18–61 months). All the patients underwent reconstruction

by a standardized L-shaped rib cartilage graft to restore the

nasal framework using an external rhinoplasty approach.

One patient (case 1) with WG had previously undergone

R0 tumor resection of a squamous cell carcinoma, which

had resulted in the destruction of her nasal architecture.

Nasal reconstruction using an L-shaped graft was per-

formed 2 years later without complications in the presence

of high-dose immune suppression related to WG and liver

transplantation (Table 1).

A second patient (case 2), who married 10 months after

L-graft nasal reconstruction, changed her previous immune

suppressive medication of anti-tumor necrosis factor-a(anti-TNF-a) to azathioprin and prednisolone because she

became pregnant. After delivery without complications, the

patient noticed recurrence of WG because of nasal crusting

and bleeding. Therefore, immunosuppressive therapy was

again changed to anti-TNF-a and mycophenolatmofetil.

Despite this, no resorption of the cartilage, nasal instability,

or other complication was observed during a follow-up

period of 26 months (Table 1).

For a third patient (case 3, Fig. 1a–d), with an open

defect of the maxilla and the paranasal sinus, a local

turnover flap was performed for reconstruction of the

mucosa and the medial canthus with simultaneous tissue

reconstruction using a cheek rotation flap according to

Esser as a first stage. The subsequent reconstruction of the

Aesth Plast Surg

123

nasal dorsum was performed 18 months later under

immune suppression with methotrexate and prednisolone.

The patient experienced a stable postoperative result over a

period of 1� years (Table 1).

The fourth patient (case 4, Fig. 2a–j) was a 26-year-old

woman with prednisolone 10 mg/day as immunosuppres-

sant therapy during the perioperative period. The latest

follow-up visit 5 years after nasal reconstruction showed

an unaltered appearance with a pleasing result (Table 1).

Discussion

The incidence of WG increases with age, peaking at ages

45 to 60 years, and ranges from 4.9 per million population

in Spain to 10.5 per million population in Norway. This

difference between the North and South is also seen in the

prevalence. Even in Germany, it varies between the

northern (58 per million) and southern (42 per million)

parts of the country [8].

This disorder of still unknown origin classically presents

with a triad of pulmonary, renal, and upper-airway mani-

festations among a wide spectrum of potential involvement

of nearly all organ systems [7] Head and neck complica-

tions are the most common clinical manifestations at pre-

sentation followed by pulmonary, orbital, and renal

involvement. The nose and paranasal sinuses are the most

frequently affected sites in the head and neck [9].

Patients may report foul-smelling rhinorrhea, epistaxis,

hyposmia, or epiphora, and clinical findings range from

mild nasal obstruction and pain over the nasal dorsum to

total nasal collapse. The nasal exam classically shows nasal

crusting, mucosal inflammation, granulation tissue, and

evidence of sinusitis. Chronic sinusitis occurs in approxi-

mately 50% of those with nasal involvement. Destruction of

the nasal septum resulting in a saddle-nose deformity may

occur in up to 23% of those with nasal involvement [2].

External nasal deformity has a devastating psychologi-

cal effect on a patient affected by WG because destruction

of the cartilaginous nasal framework may lead to saddle-

nose deformity, which has historically been a stigma of

Fig. 1 a,b A 30-year-old patient with saddle-nose deformity and

sinus fistula of the right maxilla. c,d Results 18 months after open

rhinoplasty using a costal cartilage L-strut for reconstruction of the

nasal framework. The open defect of the maxillary sinus was

reconstructed with a cutaneous turnover flap for inner mucosa

restoration. External coverage was created by a cheek skin rotation

flap

Table 1 Patient characteristics

Patient Age

(years)

Gender Autoimmune

disease

Reconstruction Immunsuppressiona Additional Follow-up

(months)

1 42 W Wegener

granulomatosis

L-shaped rib

cartilage graft

MMF 3 9 250 mg/day ?

prednisolone 10 mg/day

Liver transplantation ?

squamous cell carcinoma

of nasal septum

58

2 34 W Wegener

granulomatosis

L-shaped rib

cartilage graft

Infliximab 350 mg/8 week ?

prednisolone 10 mg/day

26

3 30 W Wegener

granulomatosis

L-shaped rib

cartilage graft

Methotrexate 25 mg/week ?

prednisolone 7.5 mg/day

Open defect of sinus

maxillaris

15

4 26 W Wegener

granulomatosis

L-shaped rib

cartilage graft

Prednisolone 10 mg/day 61

MMF mycophenolatmofetila Immunsuppression at time of operation

Aesth Plast Surg

123

other diseases such as syphilis or leprosy. Common char-

acteristics of this deformity include depression of the

middle vault and dorsum, lost definition or overrotation of

the nasal tip, shortened vertical nasal length, and retrusion

of the nasal spine and caudal septum [5, 6].

The issue of reconstructing the nose, which is the most

important aesthetic feature of the central face, often has

been neglected in the treatment of patients with WG [1, 4, 6,

15]. This underutilization of reconstructive rhinoplasty for

patients with inflammatory conditions such as WG may be

due to various reasons. First, the main focus is to cure a life-

threatening disease and not to remedy an aesthetic defor-

mity. However, as modern pharmacologic treatments have

increased the survival rate and life expectancy of these

patients dramatically, this formally subordinate aspect of

facial stigmatization has gained new importance [15].

Furthermore, most of these patients are treated by non-

surgical colleagues who may be less aware of the recon-

structive techniques available. The low numbers of these

patients in the literature and the lack of review articles may

be the reason for the scepticism of surgical colleagues as

well as their concerns about graft resorption and surgical

failure. Some physicians may hesitate to reconstruct these

defects fearing that surgery may result in a flare-up of the

WG both locally and systemically or that recurrence of the

disease, reported to be as high as 50%, may result in ulti-

mate failure of the reconstruction [4, 8].

Among our patients, no perioperative recurrence of WG

either locally or systemically occurred within a median

follow-up period of 42 months. The question whether the

patient must be in remission with minimal or no local

disease at the time of surgery remains unanswered because

all of the patients who underwent surgery had either mild

or no symptoms at the time of the reconstruction. This

philosophy requiring remission is shared by other authors

because disease severity did seem to influence the outcome,

although statistically significant conclusions could not be

drawn due to the limited number of cases in the literature

[4, 15].

Congdon et al. [4] reported a success rate of 60% for the

‘‘severe’’ WG group versus 88% for the ‘‘limited’’ WG

group. In their series, treatment using irradiated homografts

Fig. 2 a,b A 26-year-old woman with saddle-nose deformity.

c L-shaped costal cartilage strut. d,e Insertion and positioning of

the L-strut for nasal framework reconstruction. f,g Results 5 months

after open rhinoplasty and L-span reconstruction of the nasal

framework. h,j Results 5 years after open rhinoplasty and L-span

reconstruction of the nasal framework

Aesth Plast Surg

123

failed, which underscores the advantage of using living

autogenous materials such as costal cartilage or calvarial

bone, especially for those patients who present with com-

promised vascularity.

Identifying the components of the saddle-nose defect

that require reconstruction in a deformed nose is critical,

particularly in complex defects. A WG nasal deformity

may include deficits in external coverage, support, and

nasal lining.

A variety of techniques have been described for recon-

struction of saddle-nose deformity, including forehead

flaps and nasolabial flaps, which can be used for both

external coverage and nasal lining [12], yet controversy

exists with respect to the choice of method in WG [13, 14].

Some authors have stated that any reconstructive attempts

should be completely extramucosal because the heavily

contaminated nasal crusting could lead to infection of the

cartilaginous or bony replacement of the dorsum. Other

authors favor importing vascularized tissue because nasal

lining would minimize the risk of infecting the cartilagi-

nous strut. The disadvantage of this approach is that it may

be technically demanding, and it requires at least two

separate operations.

We chose a less invasive one-stage procedure to

reconstruct only the nasal framework using an L-shaped rib

cartilage graft. Theoretically, reconstruction of the inner

lining with mucosa-like tissue may improve a humidified

nasal passageway and decrease the amount of ozena and

crusting frequently associated with WG [6]. However, our

patients also experienced good aesthetic and functional

results, with improved breathing despite restoration of only

the external nasal skeleton.

Overall, neither in our own small group nor in any other

patient group in the literature did any adverse effect occur

after the primary or secondary rhinoplasty for saddle-nose

deformity in WG. The published success rate for dorsal

augmentation varied by study but usually exceeded 90%

[4]. Because revision rates for patients with WG under-

going external nasal reconstruction seem to be higher than

with normal septorhinoplasty, preoperative counseling

about this fact is warranted [4].

Because almost every patient achieved a favorable final

result without major adverse sequelae, nasal reconstruction

in WG patients using a single L-strut seems to be a safe and

worthwhile procedure, even if only the external framework

is restored without the inner lining.

Conclusion

Saddle-nose deformity due to WG is a highly stigmatizing

external sign of disease, yet regrettably, only a few

patients receive aesthetic reconstruction due to concerns

of failure caused by recurrent inflammation, infections,

and wound-healing problems. According to this patient

series and a review of the literature, external nasal

reconstruction in patients with WG appears to be safe and

effective if the disease is in remission before reconstruc-

tion is started. Although high-dose systemic steroid ther-

apy may increase the risk of failure in primary nasal

dorsal repair for WG patients compared with other

patients, the procedures seem justified to improve the

physical appearance and thus the physiologic well-being

of these chronically ill patients. Every patient with saddle-

nose deformity should be informed about the possibility

of surgical reconstruction.

Conflicts of interest The authors state that there is no source of any

financial or material support and that they don’t have any commercial

interests in the subject of study.

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