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.     

QT间期延长综合征与麻醉

QT间期延长综合征(long QT syndrome,LQTS)是由于离子通道变异,可引起致死性心律失常的一类疾病.围术期是LQTS的高危时期.此文总结了各种麻醉药物对QT间期的影响,以及LQTS患者围术期处理的注意事项,但尚不能确定最合理的麻醉方法,未来的研究应着重于手术期间的生理和药理对跨壁复极离散度的影响.     

预激综合征根治术的麻醉处理

预激综合征根治术的麻醉处理孙红，邓硕曾本院自１９８９年～１９９１年间共行预激综合征（ＷＰＷ）根治术１８例，现将其麻醉处理特点及体会报道如下。临床资料１８例病人中男性１２例，女性６例。年龄１４～５０岁，平均２７．２２±０．１６岁。体重３６～７１ｋｇ，平...     

干燥综合征患者的麻醉管理

干燥综合征(s(o)gren's syndrome,SS)是一种淋巴细胞侵犯外分泌腺体,特别是泪腺、唾液腺为主的慢性自身免疫性疾病.患者全身状况一般较差,病情复杂.对于1例53岁女性干燥综合征15年患者,拟行左股骨粗隆间骨折复位内固定术.作者在充分熟悉SS的病理生理及缓解症状的药物基础上,术前重视补钾改善全身状况,插管时避免损伤气管、支气管黏膜,术中及时监测血气及电解质,术中及术后应用氢化可的松预防皮质醇危象,保证了患者安全度过围手术期.     

直立性心动过速综合征：产科麻醉处理的思考

我们报道一例患有直立性心动过速综合征产妇的麻醉处理，这种综合征表现为血流动力学不稳定，并可能随产程及分娩生理的变化而恶化。我们讨论对患有此类疾病产妇的麻醉相关问题，并提出麻醉处理的方法。     

预激综合征根治术的麻醉处理

预激综合征根治术的麻醉处理梁韶军１岳云１郑强荪我院自１９８９年至１９９３年对预激综合征２６例患者施行了根治术。现总结其麻醉处理的经验。临床资料２６例中，男１１例，女１５例，年龄５～６３岁，其中≤１４岁５例，１５～６０岁１９例，≥６１岁２例。隐匿性预激...     

Anesthetic management of maternal Mirror syndrome

Mirror syndrome (Ballantyne syndrome, triple edema, maternal hydrops, pseudotoxemia) is a rarely diagnosed condition associated with pregnancy that can be life-threatening for both the mother and fetus. There is limited literature on its pathogenesis and anesthetic management, making prevention and treatment complex. The duration of pregnancy and severity of maternal or fetal presentation often determines outcome. We describe the anesthetic considerations of a morbidly obese parturient with Mirror syndrome.     

Lowe's syndrome with Fanconi syndrome for ocular surgery: perioperative anesthetic considerations

Lowe's syndrome is a rare inherited metabolic disorder characterized by mental retardation, kidney malfunction, and abnormalities of the eyes and bones. A 4 month-old child with Lowe's and Fanconi's syndrome, undergoing bilateral congenital cataract surgery, is presented. Preoperative electrolyte imbalance was corrected by potassium, calcium, magnesium, phosphate, and bicarbonate supplementation. Anesthesia was administered uneventfully using appropriate anesthetic agents and monitoring. Adequate preoperative evaluation and optimization, along with selection of anesthetic agents and fluid and electrolyte management with appropriate perioperative monitoring, is key to a successful outcome.     

Anesthetic management of patients with Melkersson Rosenthal syndrome

Melkersson Rosenthal Syndrome (MRS) is a rare disorder characterized by relapsing facial paralysis, persistent or recurrent orofacial edema, and lingua plicata. It may cause difficult airway, drug allergy, and angioedema. In our anesthetic management of two patients with MRS, preanesthetic immunological blood examination and skin tests for hypersensitivity to anesthetic drugs were applied. Because the principal goal is to avoid all factors that may stimulate, an allergic reaction, anesthetic drugs known to trigger urticaria were avoided. Body and operating room temperatures, changes of which may trigger allergic reactions, were kept constant during the perioperative period. Emergency precautions were taken for probable angioedema. MRS is a rare syndrome, and if its manifestations are misunderstood as simple facial paralysis, it may be overlooked by anesthesiologists. Anesthesiologists must be careful of several problems in patients with MRS.     

Leigh syndrome: anesthetic management in complicated endoscopic procedures

BACKGROUND: Leigh's syndrome, a disorder of infancy and childhood, is characterized by gray matter degeneration and focal brainstem necrosis. It presents with special clinical features such as developmental delay, nervous system dysfunction, respiratory abnormalities, and hypertrophic cardiomyopathy that can be a real challenge to the anesthesiologist. Anesthesia or sedation has rarely been reported in patients with Leigh disease. We report our experience in sedating five children with Leigh syndrome in seven procedures undertaken in the endoscopy suite (outside the operating room). METHODS: Five children with Leigh disease, three girls and two boys, have been referred to us for percutaneous endoscopic gastrostomy (PEG) insertion and or replacement (a total of seven procedures).The average age was 2.6 years with a range of 4 months to 6 years. Informed consent was obtained from the patient's parents or guardian. An anesthesia machine, scavenging system, O(2) source and routine monitoring were available. Sedation was accomplished with propofol intravenous (i.v.) (0.5-1 mg x kg(-1)) maintained with a propofol infusion (50-100 microg x kg(-1) x min(-1)). The spontaneously breathing patients received oxygen through an oxygen facemask during the procedure and afterwards recovery was managed in the gastroenterology unit. RESULTS: All the children underwent the procedure without complications. One patient developed transient desaturation (SpO(2) 80%) for a few seconds. Body temperature, heart rate, arterial blood pressure, O(2) saturation and endtidal CO(2) were stable during the endoscopies. No special post-procedure management was required; the patients woke up at the end of the endoscopy and were able to drink and eat as usual. CONCLUSIONS: This rare mitochondrial disease presents unique management problems to the anesthesiologist when using general anesthesia. Our patients were managed appropriately before endoscopy and underwent the procedure under deep sedation. No complications occurred. We concluded that deep sedation in the endoscopy suite was safe in this small series of patients with this rare disease.     

Alagille syndrome and pregnancy: anesthetic management for cesarean section

A 34-year-old multiparous woman with a breech presentation, intrauterine growth restriction and premature rupture of membranes was transferred to our referral unit at 33 weeks of gestation. She was diagnosed with Alagille syndrome soon after birth because of cholestasis and pruritus. Her condition was later complicated by esophageal varices, treated with propranolol, thrombocytopenia, and insulin-dependent diabetes. She had characteristic facies, posterior embryotoxon, “butterfly” vertebrae but had no cardiac or renal abnormalities. Due to the early onset of spontaneous labor, emergency cesarean section under general anesthesia was performed 48 h after admission. This is the first case describing anesthetic care during delivery in a patient with Alagille syndrome. We discuss the anesthetic implications of the syndrome, emphasizing problems associated with portal hypertension and cholestasis, thrombocytopenia and cardiac abnormalities such as pulmonary artery stenosis.