Laryngotracheal stenosis requiring emergency tracheostomy as the first manifestation of childhood-relapsing polychondritis.

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    • Abstract:
      Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory, recurrent and destructive cartilage lesions. The chondritis could be widespread and involves generally laryngeal and auricular hyaline cartilages. We described a 9 years and 4 months old girl, who presented recurrent acute laryngotracheitis and laryngotracheal stenosis, which were the first manifestations of relapsing polychondritis, and was submitted to emergency tracheostomy. She also had ear condritis and arthritis, being treated with prednisolone and methotrexate. In conclusion, we reported a rare case of relapsing polychondritis that presented a fife-threatening laryngo-tracheo-bronchial disorder requiring tracheostomy. We suggest that the diagnosis of relapsing polychondritis should be considered for patients who present recurrent acute laryngotracheitis with other types of condritis, as well as musculoskeletal manifestations. [ABSTRACT FROM AUTHOR]
    • Abstract:
      A policondrite recidivante é uma doença rara na infância, de etiologia desconhecida, caracterizada por lesões inflamatórias, recorrentes e destrutivas de cartilagem. A condrite pode ser generalizada e envolve principalmente as cartilagens hialinas laríngea e auricular. Relatamos uma menina de 9 anos e 4 meses de idade, que apresentou laringotraqueítes agudas recorrentes e estenose laringotraqueal como primeira manifestação de policondrite recidivante, e foi submetida à traqueostomia de emergência. Apresentou também condrite auricular e artrite, sendo tratada com prednisona e metotrexato. Concluindo, relatamos um caso raro de policondrite recidivante que apresentou uma complicação laringotraqueo-brônquica com risco de vida exigindo traqueostomia. Sugerimos que o diagnóstico de PR deva ser considerado em pacientes que apresentem laringotraqueítes agudas recorrentes associadas a outros tipos de condrites, assim como manifestações musculoesqueléticas. [ABSTRACT FROM AUTHOR]
    • Abstract:
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