對一軟骨發育不全性侏儒合併水腦症進行減壓手術之氣道處理

Airway Management of an Achondroplastic Dwarf with Hydrocephalus Undergoing Decompression Surgery

軟骨發育不全症是造成侏儒病最常見的原因。其神經學的問題為枕骨肥厚引起枕骨大孔狹窄造成水腦症，及第一頸椎異常造成之脊髓壓迫，而枕骨下頭顱切開術是治療的方法。我們報告一個三個月大患有軟骨發育不全症的病例，因枕骨大孔狹窄造成水腦症必須進行枕骨下頭顱切開減壓手術。病人也於恢復室清醒並順利拔管。外科醫師考量到頸椎伸展可能造成脊髓壓迫更嚴重，於是要求我們使用清醒時氣管插管，並於插管後確認病人第一頸椎下的運動功能完好。整個手術過程相當平順，我們同時回顧有關軟骨發育不全症的討論及麻醉時可能面臨到的相關問題。

The neurological problem of achondroplasia is hydrocephalus which may arise from stenosis of the foramen magnum secondary to occipital hypertrophy. The spine is also affected by abnormalities in the cervical region (odontoid hypoplasia and occipitalization of Q), which can compound the problem of cord compression. We report a rare instance of achondroplasia in a 3-month-old female infant with the risk of spinal cord compression during the manipulation of endotracheal intubation. For fear of that the surgeon inclined awake intubation in order that spinal cord intactness could be confirmed by neurological test on the spot after intubation. Awake oral endotracheal intubation was performed although with some difficulty, yet the motor function at the first cervical spinal level was unmolested as revealed by neurological test after intubation.