INTRODUCTION: Introduction: Rhinoscleroma is an important cause of nontraumatic subglottic stenosis. It is caused by the organism Klebsiella rhinoscleromatis, which is endemic in Central America, and has an affinity for the mucosa of the upper respiratory tract. Overcrowding and lower socioeconomic status are important epidemiological factors in its transmission. The disease often begins with rhinitis and progresses over years to a destructive fibrotic phase. Treatment requires a prolonged course of antibiotic.
CASE PRESENTATION: A twenty-year-old Hispanic female, who was a lifelong nonsmoker, presented with one-year history of progressive shortness of breath. Symptoms began shortly after emigrating from Mexico, with chronic rhinitis, which progressed over the subsequent year to exertional dyspnea and stridor. Flow volume loop was suggestive of fixed airway obstruction, CT scan of the neck and chest showed narrowing of the subglottic area to three centimeters secondary to circumferential thickening of the subglottic area. The CT of the chest was otherwise unremarkable. Flexible bronchoscopy showed thickened, erythematous mucosa from the level of the posterior pharynx to the subglottic area. Rigid bronchoscopy with dilatation was performed with debridement and biopsy of the subglottic tissue. Biopsy was negative for granulomatous tissue, with areas of chronic and focally acute inflammation, cultures grew Klebsiella rhinoscleromatis. The patient was subsequently lost to follow up.
DISCUSSIONS: This case illustrates the importance of recognizing rhinoscleroma as a cause of subglottic stenosis. The disease most commonly affects the nasal passages, initially leading to an exudative phase with nasal congestion, edema and suppurative necrosis. The next phase is the proliferative or granulomatous phase characterized by reddish nodules. Biopsies of the affected areas in this phase may reveal a characteristic finding in pathologic specimens known as Miculikz cells, which are vacuolated histiocytes containing the organism. The disease then progresses to the final stage, characterized by fibrosis. Granulomatous inflammation may lead to destruction of bone and cartilage in the upper respiratory tract. Curative treatment is often challenging, ciprofloxacin has been shown to have the greatest in vitro activity against Klebsiella rhinoscleromatis. Treatment should be continued for 6 months to one year. Treatment in the fibrotic stage is often supportive, and may include tracheal dilatation, laser excision, and tracheostomy.
CONCLUSION: Rhinoscleroma is an important cause of nontraumatic subglottic stenosis, and should be considered in individuals who have emigrated from endemic areas. As the number of individuals from endemic areas is increasing in the United States, this disease may be seen with increasing frequency. Clinical suspicion and early, prolonged treatment are required to prevent progression to the fibrotic stage.
DISCLOSURE: Pradeep Ramachandran, None.