[Anesthetic Management of a Patient with von Recklinghausen Disease after Surgeries for Thoracic and Spinal Neurofibroma].

Takashi Eto
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Abstract

Von Recklinghausen disease is an autosomal domi- nant neurocutaneous disorder, characterized by cuta- neous neurofibromas, accompanied by café-au-lait spots. We report a case of a 51-year-old man with von Recklinghausen disease who had been operated on for thoracic and spinal neurofibroma. Exploratory thoracotomy was performed under general anesthesia combined with epidural anesthesia. After tracheal intubation with spiral tube, one lung ventilation was performed using blocker tube. General anesthesia was maintained by inhalation of oxygen, air, and desflurane and the continuous infusion of remifentanil. The opera- tive course was uneventful. The patient emerged from general anesthesia smoothly, and was extubated safely. There was no neurological abnormality after operation. Preoperative evaluation of airway status and neuro- logical findings are essential in the anesthetic management of the patient with von Recklinghausen disease. This case suggests that we must take the complication into account for anesthetic management and select the appropriate anesthetic method by routine preoperative estimation. It is of great importance that anesthesiologists evaluate the airway status and neurological find- ings of patients with von Recklinghausen disease.

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[1例胸椎神经纤维瘤术后von Recklinghausen病的麻醉处理]。
Von Recklinghausen病是一种常染色体显性神经皮肤疾病,以表皮神经纤维瘤为特征,并伴有卡萨梅-奥莱斑点。我们报告了一例51岁的冯·雷克林豪森病患者,他曾因胸椎和脊柱神经纤维瘤而接受手术。在全麻联合硬膜外麻醉下开胸探查术。气管插管螺旋管后,采用阻断管进行单肺通气。全身麻醉通过吸入氧气、空气、地氟醚和持续输注瑞芬太尼维持。歌剧课很平淡。患者顺利脱离全身麻醉,并安全拔管。术后无神经系统异常。术前评估气道状态和神经系统检查结果对冯氏病患者的麻醉管理至关重要。本病例提示我们必须考虑并发症的麻醉处理,并通过术前常规评估选择合适的麻醉方法。麻醉医师评估冯氏病患者的气道状况和神经学表现是非常重要的。
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