Durability of Nasal Reconstruction in an Adolescent with Relapsing Polychondritis Treated with Infliximab

Sir: We report a rare case of saddle nose deformity secondary to relapsing polychondritis in an adolescent girl. She underwent nasal reconstruction with a costochondral graft. Stability of the autologous graft was observed more than 1 year postoperatively, despite one episode of disease exacerbation. A 14-year-old girl with relapsing polychondritis had been treated by the rheumatology service with chemotherapeutic agents (prednisone, methotrexate, naproxen, and infliximab) for 22 months. Multiple flares of nasal chondritis for 18 months before diagnosis had resulted in a saddle nose deformity. Severe auricular chondritis followed ear piercing. The patient's disease was recalcitrant to treatment until the introduction of infliximab, which induced remission. At the time of the operation, the patient had been in full clinical remission for 6 months, but she was still receiving treatment with infliximab (7 mg/kg every 5 weeks) and methotrexate (27.5 mg per week). She has had one episode of disease flare with nasal chondritis, but the graft reconstruction has remained intact (Fig. 1).Fig. 1.: (Above) Preoperative view. The nasal dorsum is concave, and there is an apparent cephalic rotation of the nasal tip. The concavity is present in the osseous and cartilaginous dorsum. (Below) Postoperative view. Results at 1 year indicate durability of the graft despite two episodes of clinical disease progression.Relapsing polychondritis is a rare systemic inflammatory disease that results in degenerative changes to cartilage. Clinical features vary widely. McAdam et al.1 provided a useful lexicon of clinical criteria to establish the diagnosis (Table 1).Table 1: Clinical Criteria for Relapsing PolychondritisThis report is unusual for many reasons. First, most cases of relapsing polychondritis occur between ages 40 and 60. Reports of relapsing polychondritis in the pediatric population are uncommon, with about 30 cases reported in the extant medical literature.2 Thus, it is not surprising that there are only a handful of reported cases of saddle nose deformity in children secondary to relapsing polychondritis, and in none of those cases was reconstruction attempted. Second, the literature on current surgical therapy for relapsing polychondritis offers little encouragement for surgeons to intervene with nasal reconstruction. It is possible that nasal reconstruction in this small population has been avoided because of concern about the periodic flares of inflammation that might threaten the reconstruction by specific disease targeting of the graft. The vulnerability of cartilage grafts to disease progression, as well as the clinical resilience of grafts used for reconstruction, has not been addressed. What is known about nasal involvement in relapsing polychondritis? It seems to occur approximately 27 percent of the time during the onset of disease and approximately 60 percent of the time at some point during the course of the disease.3 The actual extent of nasal involvement can vary widely, ranging from mere congestion and rhinorrhea to epistaxis to frank destruction of the nasal cartilage, resulting in the classic saddle nose deformity.4 Past approaches have not modified the disease's natural history consistently. However, with the introduction of the biologic anti–tumor necrosis and alpha agents, disease remission can be achieved with decreased morbidity. It seems logical that reconstruction be limited to selected patients whose disease is in remission, whose systemic immunosuppression is minimal, and whose psychosocial stigmatization is problematic. This report simply highlights the fact that adolescents affected by relapsing polychondritis may benefit from reconstructive surgery (correction of saddle nose deformity) using autogenous cartilage, and that the cannibalistic effects of relapsing polychondritis do not necessarily affect the cartilage used for that reconstruction when the patient is maintained on treatment with inflixinab and methotrexate. David Bell, M.D., D.D.S. Pediatric Plastic Surgery Dowain Wright, M.D., Ph.D. Pediatric Rheumatology Peter D. Witt, M.D. Pediatric Plastic Surgery Children's Hospital Central California Madera, Calif.

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