Tso A S, Chung H S, Wu C Y, Li J Y, Hong C L, Yang M W, Lui P W
Department of Anesthesia, Chang Gung Memorial Hospital, Linkou, 5 Fu-Shin St., Kweishan, Taoyuan, Taiwan, R.O.C.
Acta Anaesthesiol Sin. 2001 Dec;39(4):189-94.
Relapsing polychondritis is a rare multisystem disorder of uncertain etiology. It is characterized by recurrent and progressive destruction of both elastic and hyaline cartilages as well as connective tissue. Respiratory tract involvement is associated with high mortality and morbidity. General anesthesia may be required in these patients for tracheostomy, bronchoscopy, nasal reconstruction, aortic valve replacement, and recent tracheobronchial stenting which they usually sustain. Tracheostomy was once the most likely surgical procedure in relapsing polychondritis. However, this procedure is only effective in patients with upper subglottic involvement. In cases of extensive tracheobronchial involvement, tracheostomy is ineffective because the distant tracheal collapse below the tracheostomy is still unresolved. We would like to report a case of relapsing polychondritis with tracheobronchial involvement, who underwent an emergent tracheostomy. Tracheostomy with continuous positive airway pressure (CPAP) effectively improved her airway collapse. Herein, we also discuss the anesthetic management after review of the current literature.
复发性多软骨炎是一种病因不明的罕见多系统疾病。其特征是弹性软骨和透明软骨以及结缔组织反复进行性破坏。呼吸道受累与高死亡率和高发病率相关。这些患者通常需要全身麻醉以进行气管切开术、支气管镜检查、鼻重建、主动脉瓣置换以及近期的气管支气管支架置入术。气管切开术曾是复发性多软骨炎最常见的外科手术。然而，该手术仅对上声门下受累的患者有效。在广泛气管支气管受累的情况下，气管切开术无效，因为气管切开术下方远处的气管塌陷仍未解决。我们报告一例复发性多软骨炎合并气管支气管受累患者，该患者接受了紧急气管切开术。持续气道正压通气（CPAP）气管切开术有效改善了她的气道塌陷。在此，我们在回顾当前文献后还讨论了麻醉管理。
