Klippel -Feil综合征与麻醉

背景 Klippel-Feil综合征(klippel-Feil syndrome,KFS)临床极为少见,是一种以颈椎融合为主要特征的先天性畸形,多数患者合并有其他器官系统的异常,属于困难气道的高风险人群.目的 针对KFS的临床特点,讨论此类患者麻醉管理的特殊性.内容 综述KFS的临床表现与诊断,以及麻醉管理特点,重点讨论该类患者的气道处理.趋向 充分的麻醉前颈部畸形和气道的评估,有助于合理制定麻醉及气道处理方案,避免神经损伤,提高麻醉安全性.     

Anesthetic management of a patient with Hallermann-Streiff syndrome

Key words  airway management - difficult intubation - Hallermann-Streiff syndrome     

Anesthetic management of a pregnant patient with dengue hemorrhagic fever for emergency cesarean section

Dengue fever is the most common and widespread insect-borne viral infection worldwide. The most critical phase of this viral infection is the defervescent phase when the fever subsides but the patient develops life-threatening thrombocytopenia due to complement activation by viral antigens binding to platelets. We have outlined the anesthetic management of a 25-year-old primigravida with dengue hemorrhagic fever who needed to undergo emergency cesarean section. We also describe this illness in brief and compare the anesthetic management to that of preeclampsia and hemolysis, elevated liver enzymes and low platelet syndrome which this disease mimics.     

Anesthetic management of a patient with MERRF syndrome

There are several specific considerations regarding anesthesia in patients with mitochondrial disease. We describe the successful administration of a combined general and epidural anesthesia with sevoflurane maintenance in a patient with myoclonic epilepsy with ragged red fibers (MERRF syndrome) scheduled for surgical treatment of bilateral clubfoot.     

Anesthetic management of a patient with Freeman-Sheldon syndrome

The Freeman-Sheldon syndrome (FSS) is rare congenital myopathy and dysplasia. The musculoskeletal and soft-tissue manifestations of FSS often require orthopedic and plastic reconstructive surgery. We report a case of 19-month-old girl with FSS.     

Anesthetic management of a patient with Weill-Marchesani syndrome

Weill-Marchesani syndrome is characterized by short stature, brachydactylyl, myopia, microspherophakia, lens dislocation, glaucoma, joint stiffness, restricted articular movements and facial features. The anesthetic management of an 11-year-old-male patient with diagnosis of this syndrome is reported.     

Anesthetic management of a patient with a history of Batista procedure for dilated cardiomyopathy undergoing gastric surgery

We experienced anesthetic management for an operation to remove a hemorrhagic gastric submucosal tumor in a patient who had undergone left ventricular volume reduction (the Batista procedure) for dilated cardiomyopathy (DCM) 2 years previously. Preoperative evaluations indicated the relapse of severe DCM. Intravenous and epidural anesthesia was employed with the aid of an intraaortic balloon pump (IABP). Safe anesthetic management was achieved under the guidance of a Swan-Ganz catheter without inducing overreduction of afterload or excessive preload.     

完全性大动脉错位解剖纠治手术的麻醉管理

总结了８例完全性大动脉错位（ＴＧＡ）解剖纠治术的有关麻醉管理的若干问题。其中４例室隔完整，４例伴室缺。最小年龄１８天，最轻体重３．４ｋｇ。采用大剂量芬太尼伴以安氟醚静吸复合麻醉。５例深低温停循环，３例低温体外循环，主动脉阻断时间９７．５±２０．８７分钟，全部自动复跳，１例死于肺高压，无麻醉并发症。大剂量芬太尼可使整个麻醉和手术过程较为平稳。术毕仍应维持适当麻醉深度，以利术后血流动力学平稳。变力性药物有助于心功能恢复，常用多巴胺。体外循环中按 α稳态的理论调节ｐＨ和 ＰａＣＯ２，无一例神经系统并发症。     

Anesthetic and airway management of general anesthesia in a patient with Meckel-Gruber syndrome

Meckel-Gruber syndrome, characterized by occipital encephalocele, microcephaly, polydactyly, cleft lip or palate, mandibular micrognathism, and anatomical abnormality of the larynx and tongue, along with other associated malformations, is in the list of diseases associated with difficult airway. However, there has been no report on the management of general anesthesia and airway management for such patients. A 2-year-old girl with Meckel-Gruber syndrome was scheduled for cardioplasty and gastrostomy for gastroesophageal reflux under general anesthesia. Preoperative examination revealed obesity, microgenia, dysspondylism, proteinuria, hypoplastic kidneys, and stenosis of the anal canal. Although we anticipated some difficulty with the intubation and prepared several alternative methods for intubation, such as a bronchofiberscope and a laryngeal mask airway, tracheal intubation was completed without difficulty using conventional laryngoscopy after inhalational induction with sevoflurane. Because most patients with this syndrome die before and shortly after delivery, those who survive to some age might have less severe deformities.     

Anesthetic management of a patient with Brugada syndrome

Brugada syndrome is characterized by right bundle-branch block, ST elevation in leads V 1 through V 3 and normal QT interval. Ventricular fibrillation frequently occurs in patients with Brugada syndrome. There have been few reports of anesthetic management of Brugada patients. We managed a 47-year-old man with Brugada syndrome, who underwent hemilaminectomy under general anesthesia, without untoward cardiovascular events. Potential problems in anesthetic management of patients with Brugada syndrome are also discussed.     

Anesthetic management of a patient with West syndrome

A 21-year-old female with West syndrome was scheduled for resection of hordeolum. She had an episode of convulsion at three months of age, and was diagnosed as having West syndrome at one year of age. She had epileptic seizures twice a week in spite of administration of phenytoin, clonazepam and sodium valproate. These drugs had been administered till the morning of the surgery. After premedication with atropine 0.25 mg, anesthesia was induced with propofol (12-->10-->8 mg.kg-1.h-1). The tracheal intubation was performed with vecuronium 0.1 mg.kg-1 and anesthesia was maintained with continuous infusion of propofol 6-8 mg.kg-1.h-1 and local infiltration with 1.0% lidocaine 5 ml. We administered phenytoin to prevent epileptic seizures during the surgery. No epileptic seizures occurred perioperatively. We conclude that propofol may be useful for a patient with West syndrome, and we should be careful not to lower the threshold for convulsion during the perioperative period.     

Anesthetic management of a patient with Freeman-Sheldon syndrome

We describe a case of Freeman-Sheldon syndrome that presented some problems for anesthetic management. A 2-yr-old girl required orthopedic surgery for the bilateral lower extremities. Anesthesia was induced via a mask with oxygen (2 l.min-1), nitrous oxide (4 l.min-1) and sevoflurane (approximately 5%). Tracheal intubation by direct laryngoscopy was successfully achieved. Combined caudal epidural block was, however, avoided because spina bifida occulta was suspected. Spina bifida occulta was revealed postoperatively by X-ray. For anesthetic management of a patient with Freeman-Sheldon syndrome, the spine should be evaluated preoperatively when performing epidural/spinal anesthesia.