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卡塔格内综合征——耳鼻喉科手术的麻醉考量

Kartagener's syndrome--anaesthetic considerations for ENT surgery.

作者信息

Burduk Pawel K, Wawrzyniak Katarzyna, Kazmierczak Wojciech, Kusza Krzysztof

机构信息

Katedra i Klinika Otolaryngologii i Onkologii Laryngologicznej Collegium Medium w Bydgoszczy, UMK w Toruniu.

出版信息

Otolaryngol Pol. 2012 Jul-Aug;66(4):291-4. doi: 10.1016/j.otpol.2012.05.020. Epub 2012 May 24.

DOI:10.1016/j.otpol.2012.05.020
PMID:22890535
Abstract

Kartagener's syndrome is a rare autosomal recessive disorder presenting a triad of sinusitis, bronchicetasis and situs inversus with dextrocardia. It occurs in 50% of patients with situs inversus. The most important anesthetic implications of Kartegener's syndrome surgery are assessement of pulmonary and cardiac structure and function. We present a case of 43-year-old woman with chronic rhinosinusitis with polyps and bilateral sectetory otitis media. The chest radiograph and CT scans showed dextrocardia and situs inversus with chronic bronchitis without bronchiectasis. Spirometry showed forced expiratory volume in one second (FEV1) of 2.66 L and forced vital capacity (FVC) of 3.62 L. Electroechography showed no cardiac abnormalities with 55-60% of EF. The anesthetic implications of Kartagener's syndrome are varied. The regional or general anesthesia might be involved with sinus surgery, ear surgery, pulmonary surgery, infertility or abdominal and cardiac surgery. The main anesthetic considerations among patients with Kartagener's syndrome are related to the pulmonary function which include preoperative respiratory infections due to bronchiectasis. We should also monitor potentially occluded congenital heart diseases. Kartagener's syndrome is a rare disease and when the patient need an operation we have to consider surgery with regional or general anesthesia. The general anesthesia would be safe after complete preanaesthetic examination of the patient. The ECG, chest CT scans, spirometry and echocardiography are mandatory before the operation.

摘要

卡塔格内综合征是一种罕见的常染色体隐性疾病,表现为鼻窦炎、支气管扩张和内脏转位伴右位心三联征。它发生于50%的内脏转位患者中。卡塔格内综合征手术最重要的麻醉相关问题是评估肺和心脏的结构与功能。我们报告一例43岁女性,患有慢性鼻窦炎伴息肉和双侧分泌性中耳炎。胸部X线片和CT扫描显示右位心和内脏转位,伴有慢性支气管炎但无支气管扩张。肺功能测定显示一秒用力呼气量(FEV1)为2.66L,用力肺活量(FVC)为3.62L。超声心动图显示心脏无异常,射血分数为55%-60%。卡塔格内综合征的麻醉相关问题多种多样。区域麻醉或全身麻醉可能用于鼻窦手术、耳部手术、肺部手术、不孕症手术或腹部及心脏手术。卡塔格内综合征患者主要的麻醉注意事项与肺功能有关,包括因支气管扩张导致的术前呼吸道感染。我们还应监测潜在的先天性心脏病。卡塔格内综合征是一种罕见疾病,当患者需要手术时,我们必须考虑采用区域麻醉或全身麻醉进行手术。在对患者进行全面的麻醉前检查后,全身麻醉是安全的。手术前必须进行心电图、胸部CT扫描、肺功能测定和超声心动图检查。

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