以舌唇黏合術治療上呼吸道阻塞的皮爾羅賓氏症患者－兩病例報告

皮爾羅賓氏症是一種罕見的先天性疾病，合併有下顎發育不良、舌頭後縮與時裂等表徵。上呼吸道阻塞與餵食困難是患有此症的嬰兒在臨床上時常遇到問題。嚴重的上呼吸道阻塞通常對保守性的非手術治療無效，而需要手術介入來解除。手術術式選擇有舌唇黏合術、氣管造口術及下顎牽引性骨生成術。直至今日，手術適應症與方法及何時手術仍存在爭議。本文報告兩個皮爾羅賓氏症的病例，經評估後接受舌唇黏合術治療，得以拔除插管、改善血氧濃度及體重增加。舌唇黏合術是一個簡單的外科步驟，並少有併發症，可以成功的治療部分嚴重上呼吸道阻塞的皮爾羅賓氏症患者，當術前評估呼吸道阻塞是因為舌根部後位引起時，可以優先考慮以舌唇黏合術治療。

關鍵字

皮爾羅賓氏症；上呼吸道阻塞；舌唇黏合術

並列摘要

Pierre Robin Sequence (PRS) is a rare congenital anomaly with the characteristic features of mandibular deficiency (micrognathia, or retrognathia), glossoptosis and cleft palate. Upper airway obstruction and difficulty in feeding are the common clinical problems in the affected infants. Children with severe airway obstruction will often fail conservative nonsurgical management. Several surgical options for the management have been described, including tongue-lip adhesion (TLA), tracheostomy, and mandibular distraction osteogenesis. To date, there remain no reliable guidelines for establishing when surgical intervention is warranted in the infant with PRS. Moreover, the most appropriate procedure to perform in this setting remains the subject of considerable disagreement.This article reported two infants with PRS received tongue-lip adhesion after evaluation, and the symptoms were relieved including decannulation or extubation, improvement of oxyhemoglobin saturation and weight gain. Tongue-lip adhesion is a simple surgical procedure which can successfully relieve airway obstruction with little complication and should therefore considered a prior procedure in the management of these infants, especially with the mechanism of the posterior movement of tongue.

並列關鍵字

Pierre Robin Sequence ； upper airway obstruction ； tongue-lip adhesion

國際替代計量

以舌唇黏合術治療上呼吸道阻塞的皮爾羅賓氏症患者－兩病例報告

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