Maranhão Marcius Vinícius M, de Holanda Allana Cavalcanti Fulgino, Tavares Felipe Lira
Institute of Biological Sciences, Universidade de Pernambuco, Brazil.
Braz J Anesthesiol. 2013 May-Jun;63(3):287-9. doi: 10.1016/S0034-7094(13)70232-0.
Kinsbourne syndrome is a rare neurological disorder that primarily affects children previously healthy and aged between 6 and 36 months. It is characterized by opsoclonus (rapid, irregular, horizontal and vertical eye movements) and myoclonus that may affect trunk, limbs or face, and cerebellar ataxia. It may be considered a paraneoplastic syndrome by association with neuroblastomas, hepatoblastomas and, rarely, ganglioneuromas. The aim of this paper was to present the most relevant aspects of Kinsbourne syndrome, as well as the technique used for resection of mediastinal tumor in a child with this syndrome.
Child, 1 year and 5 months, with a diagnosis of posterior mediastinal tumor and Kinsbourne syndrome. Premedicated with oral midazolam. Anesthesia induced with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Maintenance of anesthesia with sevoflurane, nitrous oxide, fentanyl, and rocuronium. Neuromuscular block reversal with neostigmine combined with atropine. Postoperative analgesia with the use of dipyrone, morphine, and ketoprofen. Taken to the intensive care unit extubated, with stable hemodynamic and respiratory parameters. ICU discharge four days after surgery and hospital discharged on the seventh postoperative day without complications. Anatomopathological examination revealed ganglioneuroblastoma.
Kinsbourne syndrome is a rare neurological disorder. The drugs used in our patient proved safe and allowed an uneventful anesthesia. Drugs that trigger or aggravate opsoclonus and myoclonus, such as ketamine and etomidate, should be avoided in these patients.
金斯伯恩综合征是一种罕见的神经系统疾病，主要影响既往健康、年龄在6至36个月之间的儿童。其特征为眼阵挛（快速、不规则的水平和垂直眼球运动）、可影响躯干、四肢或面部的肌阵挛以及小脑共济失调。因其与神经母细胞瘤、肝母细胞瘤以及罕见的神经节神经瘤相关，故可被视为副肿瘤综合征。本文旨在介绍金斯伯恩综合征的最相关方面，以及用于一名患有该综合征儿童的纵隔肿瘤切除术的技术。
一名1岁5个月的儿童，诊断为后纵隔肿瘤和金斯伯恩综合征。术前给予口服咪达唑仑。用七氟醚、氧化亚氮、芬太尼和罗库溴铵诱导麻醉。用七氟醚、氧化亚氮、芬太尼和罗库溴铵维持麻醉。用新斯的明联合阿托品逆转神经肌肉阻滞。术后使用安乃近、吗啡和酮洛芬进行镇痛。拔管后送入重症监护病房，血流动力学和呼吸参数稳定。术后四天从重症监护病房出院，术后第七天出院，无并发症。解剖病理学检查显示为神经节母细胞瘤。
金斯伯恩综合征是一种罕见的神经系统疾病。我们患者使用的药物证明是安全的，麻醉过程顺利。这些患者应避免使用可引发或加重眼阵挛和肌阵挛的药物，如氯胺酮和依托咪酯。
