RT Journal Article
SR Electronic
T1 Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 494
OP 496
DO 10.1183/09031936.97.10020494
VO 10
IS 2
A1 O Sacco
A1 B Fregonese
A1 M Oddone
A1 A Verna
A1 E Tassara
A1 C Mereu
A1 GA Rossi
YR 1997
UL http://erj.ersjournals.com/content/10/2/494.abstract
AB Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications. An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient.