Rhinoscleroma causing upper airway obstruction

Rhinoscleroma is a chronic granulomatous condition of the respiratory tract, and is not uncommon in tropical regions; particularly, Mexico, Central America and the Middle East. A few cases have been reported in North America, primarily involving immigrants from endemic countries. The causative organism is Klebsiella rhinoscleromatis, a Gram-negative coccobacillus. Diagnosis is made on the basis of culture of the organism and the characteristic pathology of Mikulicz cells on light microscopy. The condition primarily affects the upper airway, and frequently presents with nasal discharge, nasal obstruction or frontal facial pain. Despite the term 'rhinoscleroma', there may be involvement of the entire respiratory tract. Although the condition is slowly progressive, its natural course portends extensive destruction. Laryngotracheal involvement occurs in approximately 15% to 80% of cases, but patients rarely present with isolated laryngotracheal disease. In the present paper, a case of rhinoscleroma presenting with symptoms of upper airway obstruction is described. A 56-year-old male pharmacist, originally from Egypt, presented with a two-month history of shortness of breath on exertion. He complained of a foreign-body sensation in his throat with associated wheezing but denied chest pain, cough, hemoptysis or hoarseness. He had not experienced fever, weight loss or night sweats. His past medical history was significant for chronic sinusitis since childhood, characterized by long-standing malodourous secretions, crusting and intermittent nasal blockage. He had seen an otolaryngologist and received a tentative diagnosis of immotile cilia syndrome 12 years before his current presentation, although this diagnosis was not proven by biopsy and he was the natural father of two children. He had both hypertension and hyperlipidemia, and no known family history of respiratory illness or malignancy. He quit smoking six weeks before presentation and had a history of one pack/week for 20 years. On physical examination, he appeared well with no evidence of respiratory distress at rest. His blood pressure was 140/90 mmHg. His trachea was midline and he did not have cervical or supraclavicular adenopathy, cyanosis or clubbing. Examination of his chest revealed symmetric chest expansion with normal breath sounds throughout all lung fields, and specifically, an absence of stridor or wheezing. The remainder of his physical examination was normal. A chest x-ray taken at the time of presentation was normal, with no evidence of a mass or parenchymal disease. He had had an x-ray six years previously which appeared the same. He underwent spirometry and produced a flow-volume loop demonstrating classical features of fixed upper airway obstruction A computed tomography scan of the patient's thorax revealed normal lungs with wall thickening of the proximal trachea and an irregular-appearing endoluminal surface at the level of the thyroid. A subsequent computed tomography scan of the head and neck showed moderate circumferential narrowing of the subglottic larynx and superior trachea, as well as nodular thickening at the level of the inferior cricoid resulting in 50% to 60% endoluminal narrowing The patient underwent bronchoscopy, the findings of which were suggestive of a proximal tracheal tumour involving ©2005 Pulsus Group Inc. All rights reserved CASE REPORT the glottis and subglottis. The patient was referred to an otolaryngologist for evaluation of a potential malignancy. Laryngoscopy confirmed that there was a 3 cm tumour of the tracheal lumen, with evidence of cartilaginous destruction. The mid-and distal trachea were clear, as were the supraglottic and glottic larynx. Endoscopic resection was performed to improve the patient's airway and biopsies were sent for pathology. Pathology revealed a packed mucosal infiltrate of histiocytes admixed with inflammatory cells (mainly plasma cells). Silver methenamine and Gram staining revealed numerous Gram-negative bacilli within the histiocytes. The low-power view of the laryngeal biopsy showed an infiltrate of large, vacuolated histiocytes or Mikulicz cells Culture of the tumour specimen confirmed Klebsiella rhinoscleromatis was the causative organism. The patient was started on ciprofloxacin hydrochloride 500 mg twice a day for six weeks. He remained well after resection with no symptoms of upper airway obstruction. Bronchoscopic follow-up at six months demonstrated no recurrence of disease

Rhinoscleroma Causing Upper Airway Obstruction

Rhinoscleroma is a chronic granulomatous condition of the respiratory tract, and is not uncommon in tropical regions; particularly, Mexico, Central America and the Middle East. A few cases have been reported in North America, primarily involving immigrants from endemic countries. The causative organism is Klebsiella rhinoscleromatis, a Gram-negative coccobacillus. Diagnosis is made on the basis of culture of the organism and the characteristic pathology of Mikulicz cells on light microscopy. The condition primarily affects the upper airway, and frequently presents with nasal discharge, nasal obstruction or frontal facial pain. Despite the term 'rhinoscleroma', there may be involvement of the entire respiratory tract. Although the condition is slowly progressive, its natural course portends extensive destruction. Laryngotracheal involvement occurs in approximately 15% to 80% of cases, but patients rarely present with isolated laryngotracheal disease. In the present paper, a case of rhinoscleroma presenting with symptoms of upper airway obstruction is described

Rhinoscleroma Causing Upper Airway Obstruction

Rhinoscleroma is a chronic granulomatous condition of the respiratory tract, and is not uncommon in tropical regions; particularly, Mexico, Central America and the Middle East. A few cases have been reported in North America, primarily involving immigrants from endemic countries. The causative organism is Klebsiella rhinoscleromatis, a Gram-negative coccobacillus. Diagnosis is made on the basis of culture of the organism and the characteristic pathology of Mikulicz cells on light microscopy. The condition primarily affects the upper airway, and frequently presents with nasal discharge, nasal obstruction or frontal facial pain. Despite the term 'rhinoscleroma', there may be involvement of the entire respiratory tract. Although the condition is slowly progressive, its natural course portends extensive destruction. Laryngotracheal involvement occurs in approximately 15% to 80% of cases, but patients rarely present with isolated laryngotracheal disease. In the present paper, a case of rhinoscleroma presenting with symptoms of upper airway obstruction is described.Peer Reviewe