Severe respiratory dysfunction in a patient with relapsing polychondritis : should we treat the saddle nose deformity?

Summary We report a case of surgical correction of a saddle nose deformity, causing severe ventilation restrictions in a 42-year-old man diagnosed with relapsing polychondritis. Relapsing polychondritis is an autoimmune disorder, in which antibodies to type II collagen cause an inflammatory destruction of cartilage. If septal cartilage of the nose is involved, destruction leads to collapse of the dorsum of the nose, causing a saddle nose deformity. Patients suffer from a ventilation disorder of varying degree depending on the response to or onset of immunosuppressive therapy. In the described patient, the destruction of the nasal septum, in addition to unstable tracheal cartilage, caused a severe restriction in ventilation, with total collapse of the internal nasal valves during forced inspiration. To improve the function of the external airways the patient underwent surgery to reconstruct the nasal septum. Although cartilage grafts are the state of the art to reconstruct the nasal septum, we used a bone graft from the iliac crest, because the autoimmune polychondritis precludes cartilage grafting due to expected cartilage destruction. At follow up 2 years postoperatively no signs of bone resorption or deterioration of the improved airway were observed. We conclude that the use of bone grafts is a promising method to restore and improve ventilation disorders caused by a saddle nose deformity in relapsing polychondritis.