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丹迪-沃克畸形的治疗选择。

Treatment options for Dandy-Walker malformation.

作者信息

Mohanty Aaron, Biswas Arundhati, Satish Satyanarayana, Praharaj Shanti Shankar, Sastry Kolluri V R

机构信息

Department of Neurosurgery, National Institute of Mental Health and Neuro Sciences, Bangalore, India.

出版信息

J Neurosurg. 2006 Nov;105(5 Suppl):348-56. doi: 10.3171/ped.2006.105.5.348.

DOI:10.3171/ped.2006.105.5.348
PMID:17328256
Abstract

OBJECT

The aim of this study was to assess the efficacy of various treatment options available for children with Dandy-Walker malformation (DWM) and to evaluate the role of endoscopic procedures in the treatment of this disorder.

METHODS

The authors conducted a retrospective review of 72 children who underwent surgical treatment for DWM during a 16-year period. All patients underwent computed tomography scanning, and 26 underwent magnetic resonance (MR) imaging. The initial surgical treatment included ventriculoperitoneal (VP) shunt placement in 21 patients, cystoperitoneal (CP) shunt placement in 24, and combined VP and CP shunt insertion in three. Twenty-one patients underwent endoscopic procedures (endoscopic third ventriculostomy [ETV] alone in 16 patients, ETV with aqueductal stent placement in three, and ETV with fenestration of the occluding membrane in two). Three patients underwent membrane excision via a posterior fossa craniectomy. In the 26 patients who had undergone preoperative MR imaging, aqueductal patency was noted in 23 and aqueductal obstruction in three. These three patients underwent placement of a stent from the third ventricle to the posterior fossa cyst in addition to the ETV procedure. During the follow-up period, 12 patients with a CP shunt and four with a VP shunt experienced shunt malfunctions that required revision. Four patients with a CP shunt also required placement of a VP shunt. In addition, five of the 21 ETVs failed, requiring VP shunt insertion. A reduction in ventricle size noted on postoperative images occurred more frequently in patients with a VP shunt, whereas a reduction in cyst size was more appreciable in patients with a CP shunt. Successful ETV resulted in a slight decrease in ventricle size and varying degrees of reduction in cyst size.

CONCLUSIONS

Endoscopic procedures may be considered an acceptable alternative in children with DWM. The authors propose a treatment protocol based on preoperative MR imaging findings of associated aqueductal stenosis.

摘要

目的

本研究旨在评估丹迪-沃克畸形(DWM)患儿现有各种治疗方案的疗效,并评估内镜手术在该疾病治疗中的作用。

方法

作者对16年间接受DWM手术治疗的72例患儿进行了回顾性研究。所有患者均接受了计算机断层扫描,26例接受了磁共振(MR)成像。初始手术治疗包括21例患者行脑室腹腔(VP)分流术,24例行囊肿腹腔(CP)分流术,3例行VP和CP联合分流术。21例患者接受了内镜手术(16例单独行内镜下第三脑室造瘘术[ETV],3例行ETV并放置导水管支架,2例行ETV并切开阻塞膜)。3例患者通过后颅窝颅骨切除术进行膜切除。在26例术前行MR成像的患者中,23例导水管通畅,3例导水管阻塞。这3例患者除接受ETV手术外,还从第三脑室向后颅窝囊肿放置了支架。在随访期间,12例CP分流患者和4例VP分流患者出现分流故障,需要进行修复。4例CP分流患者还需要放置VP分流管。此外,21例ETV中有5例失败,需要插入VP分流管。术后影像显示脑室大小缩小在VP分流患者中更常见,而囊肿大小缩小在CP分流患者中更明显。成功的ETV导致脑室大小略有减小,囊肿大小有不同程度的缩小。

结论

内镜手术可被视为DWM患儿可接受的替代治疗方法。作者根据术前MR成像发现的相关导水管狭窄情况提出了一种治疗方案。

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