1/8. Laryngotracheal involvement of relapsing polychondritis in a Korean girl.We describe a young girl presenting with acute dyspnea because of laryngotracheal involvement of relapsing polychondritis (RP). RP, a multisystemic disorder of unknown etiology, is a very rare disease in children. It is characterized by inflammation and destruction of the cartilaginous structure of many organs, including the respiratory tract. Early respiratory tract involvement in younger patients is the greatest threat to life, and aggressive therapy, including tracheostomy and intravenous high-dose steroids, is advocated.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Anaesthetic and airway management in a child with Hanhart's syndrome.Hanhart's syndrome (oromandibular-limb hypogenesis syndrome) is a rare disease characterized by hypoglossia/aglossia, various distal limb defects and micrognathia. Difficult airway due to micrognathia may complicate anaesthetic management in this syndrome. We describe the anaesthetic management of a child with Hanhart's syndrome undergoing plastic reconstructive surgery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Relapsing polychondritis: reversible airway obstruction is not always asthma.Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Initial presentation and fatal complications of linear iga bullous dermatosis in the larynx and pharynx.Two cases of linear iga bullous dermatosis initially presenting as ulcerative lesions in the larynx and pharynx are reported. It was difficult to diagnose and treat the lesions, but they were finally diagnosed from the histopathological findings of accompanying skin lesion specimens. One of the patients required a tracheostomy due to increased airway stenosis by a laryngeal lesion. Despite general corticosteroid administration this could not be completely resolved, although partial opening of the glottis was observed, and the patient died of accidental tracheostomy tube complications during home care. Although there are no reports of this disease in the otolaryngological field, these rare diseases involving the skin and entire body should be considered in the differential diagnosis of laryngeal and pharyngeal ulcerative lesions, including airway stenosis. Furthermore, simple and safe procedures for relieving airway stenosis should be selected for rare and difficult-to-diagnose airway disease, prior to the final diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Tenderness over the hyoid bone can indicate epiglottitis in adults.adult acute epiglottitis is a rare but life-threatening disease caused by obstruction of the airway. The symptoms and signs of this disease may be nonspecific without apparent airway compromise. We encountered 3 consecutive cases of adult patients with this disease in a single 5-month period in one physician's office. In all cases, physical examination revealed tenderness of the anterior neck over the hyoid bone. These observations assisted us in identifying this rare disease quickly. We suggest that tenderness over the hyoid bone should raise suspicion of adult acute epiglottitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. Ligneous conjunctivitis: an ophthalmic disease with potentially fatal tracheobronchial obstruction. Laryngeal and tracheobronchial features.Ligneous conjunctivitis is a rare disease of unknown cause characterized by pseudomembranous, fibrous, woody, plaquelike deposits on the conjunctiva. The disease appears to be hereditary and/or familial. Deposits similar to those found in the eye occur in the larynx, tracheobronchial tree, nose and nasopharynx, and vagina. When these lesions occur in the larynx and tracheobronchial tree, voice change and potentially life-threatening obstruction and pulmonary disease may occur. This paper reports the findings in a child with ligneous conjunctivitis who was followed for 1 1/2 years and who had laryngeal and tracheobronchial involvement with voice change and airway obstruction. The literature is reviewed and the histopathologic findings and endoscopic findings and management of the patient are documented. Because the patient had multiple papillomata of both hands, a possible association with human papillomavirus was suspected but never confirmed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Tuberculous tracheobronchial strictures: clinicopathological features and management with the bronchoscopic carbon dioxide laser.Tuberculous tracheobronchial stricture is a rare disease entity at the present time. We report our experiences with carbon dioxide laser treatment for four patients with tuberculous strictures that were resistant to conventional management. All patients were relatively young but were debilitated by the disease. The lesions differed from other forms of benign tracheal strictures as they tended to involve the trachea and right side of the lung and were often multiple. The reasons for this are related to the organism per se and the anatomical arrangement of the tracheobronchial airway. The pathophysiology of tuberculous strictures in the tracheobronchial airway and the technique of bronchoscopic laser resection are outlined.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. airway obstruction in children due to plexiform neurofibroma of the larynx.Plexiform neurofibroma of the larynx is a rare disease. Three cases in children with airway obstruction are presented. After tumour reduction via a lateral pharyngotomy their tracheostomies could be closed. The surgical treatment of plexiform neurofibroma of the superior laryngeal nerve is discussed and conservative, subtotal resection is recommended.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |