巨脑-毛细血管畸形综合征伴脑积水：治疗选择及文献复习。
Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature.
机构信息
Neurosurgery Department, Hospital Sant Joan de Déu, Passeig de Sant Joan de Déu s/n, 08950, Esplugues de Llobregat, Barcelona, Spain.
Neurosurgery Department, Hospital de La Ribera, Alzira, Spain.
出版信息
Childs Nerv Syst. 2021 Aug;37(8):2441-2449. doi: 10.1007/s00381-021-05222-8. Epub 2021 May 28.
PURPOSE
We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus.
METHODS
We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them.
RESULTS
Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery.
CONCLUSIONS
We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.
目的
我们描述了 4 例巨脑-毛细血管畸形综合征（MCAP）伴脑积水患者，并对文献进行了回顾，以评估与该综合征相关的脑积水的最佳治疗方法。
方法
我们回顾了我们机构中与 MCAP 相关的脑积水系列病例，并对文献进行了回顾，分析了可能导致脑积水的原因以及为其提出的不同治疗类型。
结果
在接受脑室-腹腔（VP）分流术治疗的患者中，1 例因与她的综合征或先前的分流手术无关的颅脑创伤而接受了分流术的手术修正，并最终死亡，而另 1 例则在随访结束前未接受任何手术修正。我们接受内镜第三脑室造瘘术（ETV）治疗的患者症状有所改善，并且在 ETV 手术后没有出现任何与脑积水相关的并发症。
结论
我们更新了 MCAP 相关脑积水的治疗方法，并提出 ETV 作为一种有效的治疗方法，因为它似乎是一种安全的手术，并发症发生率低。
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