Hennessey Trevor A, Backman Steven B, Meterissian Sarkis H, Schricker Thomas
Department of Anesthesia, Royal Victoria Hospital, McGill University Health Center, Montreal, Quebec, Canada.
Can J Anaesth. 2007 Oct;54(10):835-9. doi: 10.1007/BF03021712.
To report a case of asystole during combined epidural and general anesthesia occurring in a patient with Nail-Patella syndrome (NPS), and to review the management and anesthetic implications of this rare genetic syndrome.
A 64-yr-old male with NPS, renal impairment and coronary artery disease presented for right hemicolectomy for colon cancer. Following initiation of surgery and during insertion of a nasogastric tube there was sudden loss of the patient's pulse oxymetry, and arterial pressure waveforms with an asystolic electrocardiogram signal. Atropine 0.6 mg i.v. was administered and after an asystolic period of 20-30 sec, myocardial activity commenced at 110 beatsxmin(-1) with return of normal vital signs and no further sequelae.
Nail-Patella syndrome can present with an array of anomalies that may be associated with perioperative complications. Glaucoma, nephropathy, vasomotor dysfunction, fragile teeth, abnormal muscle, skeletal and nerve anatomy as well as involvement of the central and/or peripheral nervous systems are common findings. In this setting it is postulated that a vasovagal reflex from esophageal stimulation by nasogastric tube placement may have caused the asystolic event. This response could have been exaggerated by the sympatholytic combination of neuraxial block, preoperative beta-blockade, and potential autonomic dysfunction secondary to NPS. Awareness of this uncommon disease and its presentation may serve to caution the anesthesiologist regarding the perioperative implications of patients with this syndrome.
报告1例患有指甲-髌骨综合征（NPS）的患者在硬膜外麻醉与全身麻醉联合应用期间发生心搏停止的病例，并回顾这种罕见遗传综合征的处理方法及麻醉相关问题。
一名64岁男性，患有NPS、肾功能损害和冠状动脉疾病，因结肠癌行右半结肠切除术。手术开始后，在插入鼻胃管期间，患者的脉搏血氧饱和度突然丧失，动脉压波形消失，心电图显示心搏停止信号。静脉注射0.6mg阿托品，在20 - 30秒的心搏停止期后，心肌活动开始，心率为110次/分钟，生命体征恢复正常，无进一步后遗症。
指甲-髌骨综合征可表现出一系列可能与围手术期并发症相关的异常情况。青光眼、肾病、血管舒缩功能障碍、牙齿脆弱、肌肉、骨骼和神经解剖结构异常以及中枢和/或周围神经系统受累是常见表现。在此病例中，推测鼻胃管放置刺激食管引发的迷走神经反射可能导致了心搏停止事件。神经轴阻滞、术前β受体阻滞剂以及继发于NPS的潜在自主神经功能障碍的交感神经阻滞联合作用可能使这种反应更加严重。了解这种罕见疾病及其表现可能有助于提醒麻醉医生注意该综合征患者的围手术期相关问题。
