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一例罕见的丹迪-沃克综合征病例。

A Rare Case of Dandy-Walker Syndrome.

作者信息

Oria Mohammad Sharif, Rasib Aziz Rahman, Pirzad Ahmad Fawad, Wali Ibrahim Khel Fahima, Ibrahim Khel Mohammad Ismael, Wardak Fazel Rahim

机构信息

Anesthesiology Department, Kabul University of Medical Sciences, Kabul, 1001, Afghanistan.

Neuropsychiatry Department, Kabul University of Medical Sciences, Kabul, 1001, Afghanistan.

出版信息

Int Med Case Rep J. 2022 Feb 15;15:55-59. doi: 10.2147/IMCRJ.S350858. eCollection 2022.

Abstract

Dandy-Walker syndrome (DWS) is a rare congenital malformation characterized by hypoplasia of the cerebellar vermis and its upward rotation and cystic enlargement of the fourth ventricle. The clinical manifestations include psychomotor retardation, ataxia and hydrocephalus. We report a case of 16-year-old female patient in Ali Abad Teaching Hospital who was suffering from unsteady gait, memory deterioration and urinary incontinence. A brain magnetic resonance imaging revealed enlarged cystic posterior fossa, dilated fourth ventricles and upward rotated cerebellar vermis which were indicating DWS. The patient prepared for planned surgical operation and a written informed consent was obtained from her parents for surgery and general anesthesia. A cystoperitoneal (CP) shunt was placed and then the patient transferred to recovery room. After recovery and hospital stay, the patient discharged with improved clinical symptoms.

摘要

丹迪-沃克综合征(DWS)是一种罕见的先天性畸形,其特征为小脑蚓部发育不全及其向上旋转,以及第四脑室的囊性扩大。临床表现包括精神运动发育迟缓、共济失调和脑积水。我们报告一例16岁女性患者,在阿里阿巴德教学医院就诊,她患有步态不稳、记忆力减退和尿失禁。脑部磁共振成像显示后颅窝囊性扩大、第四脑室扩张以及小脑蚓部向上旋转,这些表现提示为丹迪-沃克综合征。患者为 planned surgical operation 做准备,并获得了其父母对手术和全身麻醉的书面知情同意书。进行了囊肿-腹腔(CP)分流术,然后患者被转移至恢复室。恢复并住院一段时间后,患者临床症状改善后出院。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e1bd/8858011/581a6378d7e4/IMCRJ-15-55-g0001.jpg

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