多發性軟骨炎病患之麻醉處理－－病例報告

Anesthetic Management of a Patient with Relapsing Polychondritis-- A Case Report

多發性軟骨炎是一極罕見，不明原因及全身性疾病。其特徵為反覆性及進行性的破壞彈性軟骨，透明軟骨及結締組織。侵犯呼吸道時死亡率極高，之前認為氣管切除術是多發性軟骨炎侵犯呼吸道時的有效手術方式。但疾病延伸支氣管時，氣管塌陷仍有機會發生在氣管切除的下方。在此我們報告一位多發性軟骨炎病患，疾病已侵犯至氣管與支氣管，氣管切除術無法阻止呼吸道塌陷。氣管切除術加上呼吸道正壓呼吸可預防呼吸道塌陷。同時討論多發性軟骨炎的麻醉處理。

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.