Children with Mucopolysaccharidoses-Three Cases Report

黏多醣症是一群漸進性與遺傳有關的代謝異常疾病，由於特定溶好的缺乏，使用細胞內堆積了不完全代謝産物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的插管。在黏多醣症中有許多潛在的因素會造成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。從我們的經驗裏，我們學習到不要輕視黏多醣症的插管困難度。因爲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，支氣管痙攣也有可能産生。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。

關鍵字

黏多醣症；黏多醣症第一、二型；全身麻醉；氣管內置管

並列摘要

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 successfulintubation. 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.

並列關鍵字

Mucopolysaccharidoses ； Mucopolysaccharidostc Ⅰ, Ⅱ ； Anesthesia, general ； Intubation, intratracheal

國際替代計量

Children with Mucopolysaccharidoses-Three Cases Report

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