General Anesthesia in a Pediatric Patient with Leigh Syndrome: A Case Report and Literature Review

Leigh syndrome is a rare inherited neurodegenerative disorder of infancy and childhood and is characterized by developmental delay of nervous system function and respiratory abnormalities. The latter includes breathing difficulties, aspiration, hypoventilation, and apnea, and it is known that surgery and general anesthesia may cause acute exacerbations with respiratory failure. These patients may also develop episodes of lactic acidosis leading to respiratory failure and death. Owing to the rarity and life-threatening nature of the condition, however, there is no standard protocol for anesthetic management. Based on our own experience of evaluating and performing percutaneous gastrotomy under general anesthesia in a patient with Leigh syndrome, we discuss the attendant dilemmas and solutions and propose a working protocol with an integrated anesthetic plan.

關鍵字

Leigh syndrome ； general anesthesia ； anesthetic management

並列摘要

Leigh症候群是一種與粒腺體有關的罕見腦脊髓遺傳疾病，此疾病通常影響在出生3個月至2歲之間嬰幼兒。臨床上主要症狀是生長發育遲緩，中樞神經系統功能異常和呼吸系統障礙。患者如果需要手術時，所施行的麻醉，可能在麻醉中的施行與麻醉後的照顧，會因為沒有文獻討論，沒有標準流程的指引，而使病患涉入生命危險情況。我們在此報告一名1.5歲大患有Leigh症候群的幼兒，因胃造瘻而需外科手術。我們想藉此病患在手術施行中所需麻醉進行期間可能遭遇的代謝、呼吸、酸鹼中毒、麻醉葯物選擇、需注意事項做系統性分析與討論，也基於我們的經驗，融合既有文獻建立一標準麻醉處置流程。

並列關鍵字

Leigh症候群；全身麻醉；麻醉處置

國際替代計量

General Anesthesia in a Pediatric Patient with Leigh Syndrome: A Case Report and Literature Review

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