| Abstract [eng] |
Laryngeal stenosis is a rare inflammatory disease of the larynx, in which the laryngeal narrowing leads to the development of dyspnoea, cough and stridor. Excluding all possible causes, the aetiology of laryngeal stenosis remains unknown in 5% of cases and is classified as idiopathic subcostal stenosis. It is most common in middle-aged women and is often misdiagnosed as treatment-resistant asthma. One of the objectives of the International Rare Diseases Research Consortium (IRDiRC) is that all patients with a suspected rare disease should be diagnosed within one year of the onset of their symptoms, if the disease is described in the medical literature. Aim of the study. To identify and analyse the most important problems in the diagnosis of idiopathic subcostal laryngeal stenosis on the basis of the recent scientific literature. Methods. A systematic review of the literature in Medline and Cochrane databases was performed, and 9 articles were selected based on inclusion criteria. Results. Mean time from symptom onset to diagnosis of idiopathic laryngeal stenosis was 1.59 years (range 1.5-3.1 years). On average, patients were consulted by 2 specialists before diagnosis and 36% were consulted by ≥3 specialists. Dyspnoea (84-100%) and stridor (23-100%) were the most common early symptoms. The most common misdiagnosis in patients with idiopathic subglottic stenosis was asthma. Early CT scan (rho = 0.74, p < 0.001) and early pulmonary function tests (rho = 0.71, p = 0.01) were associated with a shorter time to diagnosis (20). Idiopathic subglottic stenosis was most commonly diagnosed by an otorhinolaryngologist (78%) and a pulmonologist (13%). Early presentation to an otorhinolaryngologist was associated with a shorter time to diagnosis (p < 0.0001)(20). Conclusions. The diagnosis of idiopathic subglottic stenosis should be excluded in middle-aged women who complain of dyspnoea during exercise or at rest, chronic cough and stridor. Early referral to an otorhinolaryngologist, CT scan of the neck and pulmonary function tests reduce the time to diagnosis of idiopathic submucosal stenosis. |
