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題 名 | Children with Mucopolysaccharidoses--Three Cases Report=小兒黏多醣症麻醉經驗--三病例報告 |
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作 者 | 滿恬恬; 蔡佩珊; 饒瑞泓; 鄭清榮; 柯元弼; 吳國驊; | 書刊名 | 麻醉學雜誌 |
卷 期 | 37:2 1999.06[民88.06] |
頁 次 | 頁93-96 |
分類號 | 417.663 |
關鍵詞 | 黏多醣症; 黏多醣症第一二型; 全身麻醉; 氣管內置管; Mucopolysaccharidoses; Mucopolysaccharidosis ⅠⅡ; Anesthesia; General; Intubation; Intratracheal; |
語 文 | 英文(English) |
中文摘要 | 黏多醣症是一群漸進性與遺傳有關的代謝異常疾病,由於特定溶□的缺乏,使得 細胞內堆積了不完全代謝產物glycosaminoglycans,進而引起多種器官及結締組織的病變與 變形,影響所及手術是無可避免的,而且麻醉的危險性是非常高的。我們在臨床中最常見的 危險就是困難插管。在黏多醣症中有許多潛在的因素會造成高危險性的呼吸道處理和氣管插 管。除了廣泛的軟組織擴散,口咽極可能被大舌頭和肥大的扁桃體所阻塞。而且鼻和口咽的 黏膜很容易出血和水腫。他們的脖子通常是短而僵硬;頸脊椎骨和顳下頷關節活動範圍常受限 制。從我們的經驗裡,我們學習到不要輕視黏多醣症的插管困難度。因為他們特殊的外型和 非常敏感的呼吸道,插管容易失敗,即使是成功的插管,支氣管痙孿也有可能產生。最近在 馬偕醫院我們連續接觸到三個患有黏多醣症的小孩,在此就我們的經驗與大家分享,並討論黏 多醣症病人的麻醉危險和處理。 |
英文摘要 | The mucopolysaccharidoses (MPS) are a group of inherited disorders of metabolism, with widespread, progressive involvement and derangement of many organs and tissues. Because of their disabling nature, frequent surgical intervention for the abnormality entailed is common, and is associated with a high degree of anesthetic risks perioperatively. One of the major hazards which we find clinically is airway difficulty. Multiple factors are present in the Mucopolysaccharidoses to make airway management and trachael intubation potentially hazardous. Aside from generalized infiltration and thickening of the soft tissues, the oropharynx may be obstructed by a large tongue with tonsillar hypertrophy. Also, the friable mucosa covering the nasal and oral pharynx renders these structures easily to bleed and edematous. The neck is typically short and immobile, and the cervical spine and tempromandibular joint may have a limited range of movement. From our experience, we have learned not to overlook the propensity of airway difficulty. The uniqueness of their anatomy and extremely sensitive airway often result in failed intubation and bronchospasm even after successful intubation. Recently, in Mackay Memorial Hospital we have encountered in series three pediatric cases with mucopolysaccharidoses (one Hurler and two Hunter syndromes). In this report we would like to share our experiences and to discuss the anesthetic risks and management of the MPS patients. |
本系統中英文摘要資訊取自各篇刊載內容。