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杜博维茨综合征患者的麻醉——病例报告

Anesthesia of a patient with Dubowitz syndrome -A case report-.

机构信息

Department of Anesthesiology and Pain Medicine, Korea Institute of Radiological & Medical Sciences, Seoul, Korea.

出版信息

Korean J Anesthesiol. 2010 May;58(5):495-9. doi: 10.4097/kjae.2010.58.5.495. Epub 2010 May 31.

Abstract

Dubowitz syndrome is a rare autosomal recessive disorder that leads to growth retardation (intrauterine, postnatal), mental retardation, a peculiar face, microcephaly, behavioral problems and eczema. The peculiar face of individuals with Dubowitz syndrome includes sparse hair and eyebrows, low-set ears, blepharophimosis, bilateral ptosis, a flat nasal bridge with a broad nasal root and micrognathia. Airway management of such individuals might be difficult due to craniofacial anomalies, such as micrognathia, cleft palate, tooth problems and craniocervical anomalies. In addition, anesthetic management may be complicated by other systemic illnesses. We report the uneventful anesthetic management of a 16-year-old girl with Dubowitz syndrome who underwent a total abdominal hysterectomy after a pelvic examination under general anesthesia. We report this case of Dubowitz syndrome with a review of the relevant literature.

摘要

杜氏综合征是一种罕见的常染色体隐性遗传病,可导致生长迟缓(宫内、产后)、智力迟钝、特殊面容、小头畸形、行为问题和湿疹。杜氏综合征患者的特殊面容包括稀疏的头发和眉毛、低位耳朵、睑裂狭小、双侧上睑下垂、扁平的鼻梁和宽的鼻根以及小下颌。由于颅面畸形,如小下颌、腭裂、牙齿问题和颅颈畸形,此类患者的气道管理可能较为困难。此外,麻醉管理还可能因其他全身性疾病而变得复杂。我们报告了一例杜氏综合征 16 岁女孩的麻醉管理情况,该患者在全身麻醉下进行盆腔检查后接受了全腹部子宫切除术。我们报告了这例杜氏综合征病例,并对相关文献进行了复习。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/222f/2881528/48674e84d8cf/kjae-58-495-g001.jpg

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