Reconstructive rhinoplasty using cadaver cartilage in relapsing polychondritis

Abstract

Relapsing polychondritis is a rare autoimmune disorder of unknown cause characterized by recurrent inflammation of cartilage predominantly affecting the ears, nose, and upper airway. The bridge of the nose and surrounding tissue can collapse, producing a saddle nose deformity. Nasal reconstruction is often challenging in these patients due to impaired wound healing and graft take caused by autoimmune inflammatory disease and prolonged immunosuppressant treatment. Many different reconstruction techniques like autologous rib, fascia lata, and calvarial bone grafts have been used. Herein we report the use of a cadaver cartilage graft in a 55-year-old woman with relapsing polychondritis and saddle nose deformity. Because of the characteristic chondritis of her autoimmune condition, cadaver cartilage was selected because it is antigenically different from the patient’s own cartilage, offering significant structural integrity for nasal reconstruction compared to other techniques.

Relapsing polychondritis is a rare autoimmune disorder that primarily affects cartilaginous structures of the upper airways. It is characterized by recurrent episodes of inflammation leading to progressive anatomical deformation with cosmetic and functional impairment of the auricular cartilage, nasal cartilage, and respiratory tract.¹ Patients may experience nasal stuffiness, rhinorrhea, and epistaxis. The bridge of the nose and surrounding tissue become erythematous, edematous, and tender and may collapse, producing a saddle nose deformity. The pathologic basis of the saddle nose is represented by a substantial loss of the dorsal height along with middle vault depression, internal nasal valve insufficiency, columellar retraction, loss of tip support, shortened vertical length, and overrotated tip.² Nasal reconstruction can be challenging due to impaired wound healing and impaired graft integration that is caused both by the disease and by prolonged immunosuppressant treatment.³ Several types of reconstruction techniques have been used in the correction of saddle nose deformity, including autologous rib cartilage, fascia lata, and calvarial bone grafts. Costal and calvarial bone were the most used graft materials in an analysis done by Ezzat et al in 2017.⁴ In this case report, we present a patient with a history of relapsing polychondritis in whom cadaver cartilage was used for reconstruction.

CASE DESCRIPTION

A 55-year-old woman with known relapsing polychondritis and a 40-pack-year smoking history presented with loss of nasal function. She had lost almost all the cartilage in her nose over 10 years earlier, resulting in a saddle nose deformity. She had near total nasal obstruction and presented to the clinic seeking surgical reconstruction. She also had overrotation of the nasal tip (middle valve collapse). Internally, there was no nasal septum, and the nasal vestibule was 4 mm on each side. Two MTF Biologics frozen rib grafts were selected for reconstruction, one for the dorsal onlay and another for the strut graft. One month after nasal reconstruction with cadaver rib graft, the patient was very pleased with the esthetic and functional results (Figure 1). Breathing was patent in nostrils bilaterally. At 1-year follow-up, her autoimmune condition had worsened. She gained about 40 pounds while on steroids; however, her nasal reconstruction from a cosmetic and functional standpoint was much improved. On exam, the nasal cartilage graft was stable and well integrated. The nasal passageways were widely patent and there was no evidence of a saddle nose deformity.

Figure 1.

(a) Preoperative, (b) 1-month postoperative, and (c) 1-year postoperative patient photos.

DISCUSSION

Accumulating data strongly suggest that both humoral and cell-mediated immunity play a role in the pathogenesis of relapsing polychondritis. Antibodies to type II collagen, matrilin-1, and immune complexes are detected in the serum of patients. The possibility that an immune response to type II collagen may be important in the pathogenesis has been supported in animal studies. Humoral responses to type IX and type XI collagen, matrilin-1 (noncollagenous protein present in the extracellular matrix in cartilage), and cartilage oligomeric matrix protein have been demonstrated in some patients. One study showed that rats immunized with matrilin-1 were found to develop severe inspiratory stridor and swelling of the nasal septum. The rats had severe inflammation with erosions of the involved cartilage, which was characterized by increased numbers of CD4⁺ and CD8⁺ T cells in the lesions. All had IgG antibodies to matrilin-1. A subsequent study demonstrated serum anti-matrillin-1 antibodies in ∼13% of patients with relapsing polychondritis. Cell-mediated immunity may also be operative in causing tissue injury, since lymphocyte transformation can be demonstrated when lymphocytes of patients are exposed to cartilage extracts. T cells specific for type II collagen have been found in some patients, and CD4⁺ T cells have been observed at sites of cartilage inflammation.²

In theory, the use of cartilage that is antigenically different than that of the patient can be beneficial in the reconstruction of saddle nose deformity in a patient with relapsing polychondritis. Many surgeons use autologous rib graft for reconstruction of the saddle nose but due to the nature of the patient’s autoimmune condition, patients may have a flare of their condition, resulting in autoimmune destruction of the graft. In a previous study, the costal cartilages were involved in 35% of patients with relapsing polychondritis.⁵

Calvarial bone grafts may offer improved resistance to resorption and formation of saddle nose in similar patients; however, bone grafts are too brittle for the lower third of the nose.^4,6 Although some sources cite that calvarial bone grafts have a higher resorption rate when compared to that of cartilage, it is not clear, as other sources claim that it has a lower resorption rate compared to cartilage.^6,7 Therefore, resorption rate may not be a reliable factor to use in deciding against calvarial bone grafts. Additionally, the outer table of the calvarial bone does not provide a graft of adequate thickness or length for nasal reconstruction. As a result, this method of reconstruction has fallen out of favor in recent years.

Fascia lata grafts have also been used in the reconstruction of saddle nose deformity in patients with autoimmune conditions. Although fascia lata can be beneficial in some contouring of the dorsal nose, it fails to provide the structural integrity required to reestablish function and esthetic appearance in saddle nose deformity.³ There is substantial lack of support especially with respect to cartilaginous integrity in saddle nose deformity. Functionally and esthetically, the main structural feature of a saddle nose is loss of septal support. Moreover, dermis and fascia have unpredictable absorption rates (up to 10%).⁶ Using a fascia lata graft to reconstruct this structural integrity is simply inadequate.