Imai Eriko, Kawamata Tomoyuki, Yamamoto Katsumi, Mochidome Mariko, Kawamata Mikito
Department of Anesthesiology and Resuscitology, Shinshu University School of Medicine, Matsumoto 390-8621.
Masui. 2012 May;61(5):538-41.
Maffucci syndrome is a rare syndrome characterized by multiple enchondromas and hemangiomas seen in various tissues and organs. We report anesthetic management of a 33-year-old man with multiple hemangiomas due to Maffucci syndrome undergoing removal of a pituitary adenoma. The preoperative examination revealed multiple hemangiomas in the head, neck, right upper and lower extremities, the tongue and the pharynx. In the operating room, we observed the locations of hemangiomas in the tongue and pharynx in detail using a fiberoptic bronchoscope before induction of anesthesia. Since there was a risk of rupture of a large hemangioma by using a laryngoscope, we planned fiberoptic bronchoscope-guided tracheal intubation without using a laryngoscope. After intravenous administration of propofol and rocuronium, a fiberoptic bronchoscope was orally introduced into the trachea and then the trachea was intubated with a flexible spiral endotracheal tube, preventing contact of the fiber or endotracheal tube with the hemangiomas. In addition, hemangiomas on the body surface were wrapped up with soft dressing to prevent rupture. Surgery was uneventfully completed. The trachea was carefully extubated, and bleeding from hemangiomas was not observed. Since patients with Maffucci syndrome have multiple hemangiomas, it is important to check for the presence of a hemangioma in the upper airway in anesthetic management.
马富西综合征是一种罕见的综合征，其特征为在各种组织和器官中出现多发性内生软骨瘤和血管瘤。我们报告了一名33岁因马富西综合征患有多发性血管瘤的男性患者，在接受垂体腺瘤切除手术时的麻醉管理情况。术前检查发现该患者头部、颈部、右上肢和下肢、舌头及咽部均有多发性血管瘤。在手术室，麻醉诱导前我们使用纤维支气管镜详细观察了舌头和咽部血管瘤的位置。由于使用喉镜有导致大血管瘤破裂的风险，我们计划在不使用喉镜的情况下进行纤维支气管镜引导下气管插管。静脉注射丙泊酚和罗库溴铵后，经口腔将纤维支气管镜插入气管，然后用柔性螺旋气管导管进行气管插管，避免纤维或气管导管与血管瘤接触。此外，体表的血管瘤用柔软敷料包裹以防破裂。手术顺利完成。小心地拔除气管导管，未观察到血管瘤出血。由于马富西综合征患者有多发性血管瘤，因此在麻醉管理中检查上呼吸道是否存在血管瘤很重要。
