Department of Neurological Surgery, University of Wisconsin Hospitals and Clinics, Madison, WI, USA.
Department of Pediatrics, University of Wisconsin Hospitals and Clinics, Madison, WI, USA.
Childs Nerv Syst. 2022 Jan;38(1):153-161. doi: 10.1007/s00381-021-05377-4. Epub 2021 Oct 20.
Chiari I malformation (CMI) and hydrocephalus often coexist, with no clear understanding of the cause-and-effect relationship. In the absence of other associated etiologies, the traditional teaching has been to treat the hydrocephalus first, partly to minimize the risk of cerebrospinal fluid (CSF) leak from CMI decompression in the setting of elevated ICP. We describe a series of consecutive pediatric patients with CMI and hydrocephalus, the majority of whom were managed with posterior fossa decompression.
A retrospective review was conducted on consecutive children who presented to the senior author with both hydrocephalus and CMI, with emphasis on rationale for and outcomes of surgical intervention, including the need for additional surgery.
There were 14 patients aged 2 weeks to 16 years (median 2 years) who presented with Chiari I and hydrocephalus. Four of these underwent posterior fossa decompression without duraplasty (PFD) as first-line therapy (one of whom eventually required duraplasty), 7 had PFD with duraplasty (PFDD), 1 received a ventriculoperitoneal shunt (VPS), and two had endoscopic third ventriculostomy (ETV). Of the 11 who had PFD/D, 9 (90%) had significant symptom improvement/resolution, 7 (55%) showed decrease in ventricle size, and 1 (10%) required VPS placement for persistent hydrocephalus. Both ETV patients improved clinically, and 1 showed decrease in ventricle size. There were no pseudomeningoceles, infections, or neurological deficits. One CSF leak occurred after an ETV and was successfully treated with wound revision.
In patients with both CMI and hydrocephalus, treating the CMI first in an effort to avoid a shunt can be safe and effective. In this series, PFDD in the setting of hydrocephalus did not result in CSF leak or pseudomeningocele. While limited by a small sample size, these data support a causative relationship between CMI and hydrocephalus.
Chiari I 畸形（CMI）和脑积水常同时存在，但因果关系尚不清楚。在没有其他相关病因的情况下，传统的治疗方法是先治疗脑积水，部分原因是为了降低因颅内压升高而行 CMI 减压导致脑脊液（CSF）漏的风险。我们描述了一系列连续的伴有 CMI 和脑积水的儿科患者，其中大多数患者接受了后颅窝减压治疗。
对就诊于高级作者的伴有脑积水和 CMI 的连续儿童进行回顾性分析，重点关注手术干预的理由和结果，包括是否需要额外手术。
共有 14 名年龄在 2 周至 16 岁（中位数 2 岁）的患者表现为 Chiari I 和脑积水。其中 4 例患者接受了单纯后颅窝减压术（PFD）作为一线治疗（其中 1 例最终需要行硬膜修补术），7 例患者行 PFD 加硬膜修补术（PFDD），1 例行脑室-腹腔分流术（VPS），2 例行内镜第三脑室造瘘术（ETV）。在接受 PFD/D 的 11 例患者中，9 例（90%）的症状显著改善/缓解，7 例（55%）的脑室大小减小，1 例（10%）因持续性脑积水需要行 VPS 植入。2 例 ETV 患者的临床症状均有改善，1 例脑室大小减小。无假性脑脊膜膨出、感染或神经功能缺损。1 例 ETV 后发生脑脊液漏，经伤口修复成功治疗。
在伴有 CMI 和脑积水的患者中，首先治疗 CMI 以避免分流是安全有效的。在本系列中，PFDD 联合脑积水治疗并未导致脑脊液漏或假性脑脊膜膨出。尽管样本量较小，但这些数据支持 CMI 和脑积水之间存在因果关系。
