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先天性皮肤窦道患儿的临床特征及诊治方法(附56例报告) 被引量:3

Clinical features,diagnosis and treatment of children with congenital dermal sinus:A report of 56 cases
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摘要 目的探讨先天性皮肤窦道患儿的临床特征及诊疗经验。方法回顾性分析2005年1月至2017年9月上海交通大学医学院附属上海儿童医学中心神经外科收治并手术的56例皮肤窦道患儿的临床资料。其中,47例为初发患儿,9例为复发患儿。56例患儿的中位年龄为2岁(1个月至11岁)。其中皮肤窦道位于后枕部2例,颈椎平面1例,胸椎平面7例,腰椎平面26例及骶尾椎平面20例。术前观察病变区皮肤、行头颅或脊椎MRI检查。47例初发患儿行皮肤窦道切除术,所有患儿中合并脊髓或颅内病变的患儿行病变切除术,合并拴系的患儿均行脊髓拴系松解手术治疗。采用术前、术后对照评价神经功能的变化情况。随访患儿的神经功能和影像学变化。结果56例患儿的病变区皮肤均存在"针眼样孔"。43例术前MRI提示硬膜内存在病变;另13例首次手术前MRI未提示硬膜内病变,其中9例在后期手术中发现硬膜内皮样或表皮样囊肿。92.9%(52/56)的患儿有皮肤窦道合并硬膜内皮样囊肿(33例)或表皮样囊肿(19例)。56例患儿的中位随访时间为4年(2个月至12年)。无中枢神经系统感染的46例患儿中,16例术前有神经功能损伤,术后痊愈4例,改善9例,无变化3例;另30例术前无神经功能损伤,术后神经功能亦正常。合并中枢神经系统感染的10例患儿,术前均有神经功能损害,术后8例改善,2例恶化。结论发现患儿后枕部、脊柱中线区域有"针眼样孔",应联合MRI检查明确皮肤窦道,尽早行手术治疗;对于MRI未提示硬膜内病变的患儿,手术时仍应行硬膜内探查。 Objective To discuss the clinical features,diagnosis and treatment of children with congenital dermal sinus tract.Methods A retrospective analysis was conducted on the clinical data of 56 children with congenital dermal sinus admitted to Department of Neurosurgery,Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2005 to September 2017.There were 47 primary cases and 9 recurrent cases.The median age of 56 children was 2 y(1m to 11y).The congenital dermal sinus was located in the posterior occipital region in 2 cases,cervical area in 1,thoracic area in 7,lumbar area in 26,sacral and coccygeal area in 20.The skin lesion area was observed prior to surgery.Head or spine MRI examination was undertaken.The 47 primary cases underwent resection of the dermal sinus tract.The lesions were removed for those with spinal or intracranial lesions.For those with combined tethered spinal cord syndrome,spinal cord untethering was performed.Comparison between pre-and post-operative conditions was conducted to evaluate changes in the neurological function,which,as well as medical imaging,was followed up.Results In 56 cases,there were"needle-eye-like holes"in the lesion area of the children.In 43 cases,preoperative MRI suggested intradural lesions.In the remaining 13 cases,preoperative MRI did not indicate intradural lesions,9 of which had epidural or epidermal cysts revealed later during operation.Overall,92.9%(52/56)of children had dermal sinus combined with spinal cord endothelial cyst(33 cases)or epidermoid cyst(19 cases).The median follow-up time for 56 children was 4 years(2 months to 12 years).Of the 46 cases of children without central nervous system infection,16 had neurological function damage before surgery.Among those,4 cases recovered after surgery,9 cases were improved and 3 cases remained unchanged.In 10 cases with combined central nervous system infection,there was neurological function damage before surgery.Among them,8 cases improved after surgery,and 2 cases deter
作者 夏泽阳 鲍南 杨波 宋云海 高俜娉 Xia Zeyang;Bao Nan;Yang Bo;Song Yunhai;Gao Pingping(Department of Neurosurgery,Shanghai Children′s Medical Center,Shanghai Jiao Tong University School of Medicine,Shanghai 200120,China)
出处 《中华神经外科杂志》 CSCD 北大核心 2020年第6期560-564,共5页 Chinese Journal of Neurosurgery
基金 上海市卫生和计划生育委员会重点科研项目(20124018)。
关键词 椎管闭合不全 神经外科手术 中枢神经系统感染 皮肤窦道 Spinal dysraphism Neurosurgical procedures Central nervous system infections Dermal sinus
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