1Department of Neurosurgery, Great Ormond Street Hospital for Children, London.
2Developmental Imaging and Biophysics Section, UCL GOS Institute of Child Health, London.
J Neurosurg Pediatr. 2020 Oct 23;27(1):52-61. doi: 10.3171/2020.6.PEDS2089. Print 2021 Jan 1.
The goal of this study was to characterize the complications and morbidity related to the surgical management of pediatric fourth ventricle tumors.
All patients referred to the authors' institution with posterior fossa tumors from 2002 to 2018 inclusive were screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.
Three hundred fifty-four children with posterior fossa tumors were treated during the study period; of these, 185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis. One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30 cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases), with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ventricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventriculostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%); however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS; 28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant difference in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression modeling did not reveal any statistically significant relationships between CMS and surgical approach.
Surgical management of pediatric fourth ventricle tumors continues to evolve, and resection is increasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient population.
本研究旨在描述与儿童第四脑室肿瘤手术治疗相关的并发症和发病率。
对 2002 年至 2018 年期间作者所在机构收治的所有后颅窝肿瘤患者进行筛选，仅纳入真正的第四脑室肿瘤。回顾术前影像学和临床记录，提取以下数据：首发症状；手术过程、技术和辅助手段；肿瘤组织学；以及术后并发症。
研究期间共治疗了 354 例后颅窝肿瘤患儿，其中 185 例肿瘤位于第四脑室，167 例第四脑室肿瘤具有完整数据集，纳入本分析。100 例为男性（平均年龄±标准差，5.98±4.12 岁）。最常见的首发症状为呕吐（63.5%）。最常见的肿瘤类型依次为髓母细胞瘤（94 例）>毛细胞星形细胞瘤（30 例）>室管膜瘤（30 例）>脉络丛肿瘤（5 例）> 胚胎发育不良性神经上皮肿瘤/横纹肌样瘤（4 例），另有 4 例为其他病变。在 67.1%的脑积水患者中，45.5%的患者置入了脑室-腹腔分流管（其中 66.7%在肿瘤手术前，56.9%在额部），这些患者更有可能在以后行脑室-腹腔分流术（p=0.00673）。22 例行内镜第三脑室造瘘术，其中 8 例以后行脑室-腹腔分流术。总的来说，19.7%的患者在治疗过程中安置了脑室-腹腔分流管。在整个系列中，经蚓部入路比经枕下乙状窦后入路更常见（64.1%比 33.0%）；然而，在后半部分的系列中，经枕下乙状窦后入路明显更常见（p<0.001）。全切除率为 70.7%。最常见的术后缺陷是小脑缄默综合征（CMS；28.7%），其次是新的无力（24.0%）、颅神经病变（18.0%）和新的步态异常/共济失调（12.6%）。术中超声的使用显著降低了 CMS 的发生率（p=0.0365）。在 CMS 发生率方面，枕下乙状窦后入路和经蚓部入路之间无显著差异（p=0.745），多变量逻辑回归模型也未显示 CMS 与手术入路之间存在任何统计学显著关系。
儿童第四脑室肿瘤的手术治疗仍在不断发展，越来越多的肿瘤通过枕下乙状窦后入路切除。CMS 仍然是该患者群体的主要术后并发症。
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