Total Nasal Skeletal Reconstruction Disfigured by Granulomatosis with Polyangitis (Wegener Granulomatosis)
نویسندگان
چکیده
BACKGROUND Nasal deformity is a common disorder in Wegener granulomatosis, which is produced by polyangitis of medium-sized vessels. This process may cause necrosis and destruction of the nasal mucosa and osteocartilaginous framework. At this time, there is a lack of published data on nasal deformities associated with Wegener granulomatosis. METHODS We present a 53-year-old woman with completely necrotized nasal lining and osteocartilage. The patient had a history of granulomatosis with polyangitis that was currently in remission. We reconstructed the patient's nose with fifth and sixth rib cartilages over 3 successive surgeries over a 2-year period. RESULTS During the 3-year follow-up period, neither infection nor significant cartilage graft resorption was observed, and the aesthetic result was acceptable both to the patient and her physicians. CONCLUSIONS In spite of contamination to the nasal lining and immune-compromising medications, nasal reconstruction is usually successful when considering these factors. Access to strong and abundant fifth or sixth rib cartilage presents an opportunity to reconstruct nasal destruction due to iatrogenic or autoimmune processes.
منابع مشابه
Unilateral eyelid edema and mucosal involvement as the first presentations of Wegener granulomatosis
Background: Wegener granulomatosis or granulomatosis with polyangiitis is a pauci-immune small vessel vasculitis which is usually associated with anti-neutrophil cytoplasmic antibodies (ANCA) mainly in old men. This small vessel vasculitis is usually characterized by necrotizing granulomatous inflammation with multiorgan involvement. Kidneys could be involved as the main and life-threatening co...
متن کاملWegener Granulomatosis with Oral Involvement as Primary Manifestation: A Case Study
Introduction: Wegener Granulomatosis is a rare multisystemic disease with an unknown cause, characterized by necrotic granulomatous lesions in respiratory tract, systemic vasculitis in small arteries and veins and necrotizing glomerulonephritis. Wegener can affect any organ including kidneys, eyes or other organs but classically affects upper and lower respiratory tract. One of the rare but imp...
متن کاملBronchoscopic Management of Tracheobronchial Stenosis Secondary to Granulomatosis with Polyangitis: A Case Report
Granulomatosis with Polyangitis (GPA), previously known as Wegener’s Granulomatosis, is a form of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vascuilitis predominantly involving the upper and lower respiratory tract and kidneys. Tracheobronchial involvement in GPA can have varied manifestations which include airway inflammation, mucosal granulomas, tracheobronchial stenosis, trache...
متن کاملWegener granulomatosis--rare case presentation.
Wegener granulomatosis is often misdiagnosed as pneumonia and most common cause for bilateral lung infiltrates' are bacterial, viral, pneumocystis jiroveci infection. We describe a 35 year old female with 15 days history of nasal obstruction, breathlessness and fever. Investigations in this patient revealed chronic necrotizing vasculitic and granulomatous lesion in nasal biopsy, vasculitic pauc...
متن کامل