Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6 years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality.

原发性硬膜内脊髓肿瘤患儿的脑积水极为罕见。因此，我们进行了系统的文献综述以解决流行病学、建议的病理生理机制、预后因素和此类病例的治疗。我们对儿童原发性脊髓硬膜内肿瘤表现为脑积水的病例进行了系统评价，并提供了最佳证据。根据流行病学特征（年龄、性别、肿瘤类型和位置、临床表现、存活率、传播）对患者进行定量分析。详细讨论了可能的病理生理学理论。该研究共纳入了 44 项研究，共 121 名患者。星形细胞瘤是最常见的肿瘤 (64.5%)，而大多数肿瘤位于宫颈 (31. 4%) 或颈胸区 (25.6%)。大约一半的案件涉及 6 岁以下的儿童。来自播散性肿瘤细胞的蛛网膜下腔 CSF（脑脊液）通路的阻断和由肿瘤成分引起的肿瘤性炎症被认为是主要的发病机制。通常进行手术切除肿瘤和脑积水治疗。对于病因不明的交通性脑积水患儿，应考虑原发性硬膜内脊髓肿瘤。肿瘤切除后出现脑积水与较高的死亡率有关。来自播散性肿瘤细胞的蛛网膜下腔 CSF（脑脊液）通路的阻断和由肿瘤成分引起的肿瘤性炎症被认为是主要的发病机制。通常进行手术切除肿瘤和脑积水治疗。对于病因不明的交通性脑积水患儿，应考虑原发性硬膜内脊髓肿瘤。肿瘤切除后出现脑积水与较高的死亡率有关。来自播散性肿瘤细胞的蛛网膜下腔 CSF（脑脊液）通路的阻断和由肿瘤成分引起的肿瘤性炎症被认为是主要的发病机制。通常进行手术切除肿瘤和脑积水治疗。对于病因不明的交通性脑积水患儿，应考虑原发性硬膜内脊髓肿瘤。肿瘤切除后出现脑积水与较高的死亡率有关。