Pols San Y C V, van Veelen Marie Lise C, Aarsen Femke K, Gonzalez Candel Antonia, Catsman-Berrevoets Coriene E
Departments of 1 Pediatric Neurology.
Pediatric Neurosurgery, and.
J Neurosurg Pediatr. 2017 Jul;20(1):35-41. doi: 10.3171/2017.2.PEDS16605. Epub 2017 May 12.
OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis. Tumor type, cerebellar midline location, and brainstem involvement are risk factors for pCMS that have been identified repeatedly, but they do not explain its latent onset. Ambiguous or negative results for other factors, such as hydrocephalus, postoperative meningitis, length of vermian incision, and tumor size, have been reached. The aim of this study was to identify perioperative clinical, radiological, and laboratory factors that also increase risk for the development of pCMS. The focus was on factors that might explain the delayed onset of pCMS and thus might provide a time window for taking precautionary measures to prevent pCMS or reduce its severity. The study was focused specifically on children who had undergone surgery for medulloblastoma. METHODS In this single-center retrospective cohort study, the authors included 71 children with medulloblastoma, 28 of whom developed pCMS after primary resection. Clinical and laboratory data were collected prospectively and analyzed systematically. Variables were included for univariate and multivariate analysis. RESULTS Univariate regression analysis revealed 7 variables that had a significant influence on pCMS onset, namely, tumor size, maximum tumor diameter > 5 cm, tumor infiltration or compression of the brainstem, significantly larger decreases in hemoglobin (p = 0.010) and hematocrit (p = 0.003) in the pCMS group after surgery than in the no-pCMS group, significantly more reported incidents of severe bleeding in the tumor bed during surgery in the pCMS group, preoperative hydrocephalus, and a mean body temperature rise of 0.5°C in the first 4 days after surgery in the pCMS group. Multiple regression analysis revealed that tumor size, tumor infiltration into or compression of the brainstem, and higher mean body temperature in the first 4 postoperative days were independent and highly significant predictors for pCMS. CONCLUSIONS The authors confirmed earlier findings that tumor-associated preoperative conditions, such as a maximum tumor diameter ≥ 5 cm and infiltration into or compression of the brainstem, are associated with a higher risk for the development of pCMS. Most importantly, the authors found that a 0.5°C higher mean body temperature in the first 4 postoperative days increased the odds ratio for the development of pCMS almost 5-fold. These data suggest that an important focus for the prevention of pCMS in children who have undergone medulloblastoma surgery might be rigorous maintenance of normothermia as standard care after surgery.
术后小脑缄默综合征(pCMS)在小脑肿瘤手术后的儿童中发生率为7% - 50%。典型特征包括术后1 - 2天的潜伏期、短暂性缄默、情绪不稳定以及多种运动和神经行为异常。该综合征的后遗症通常长期存在。主要病因是双侧手术损伤(无论肿瘤位置如何)小脑近端传出通路的任何组成部分,这会由于远隔性脑功能障碍导致大脑皮质区域暂时性功能障碍。肿瘤类型、小脑中线位置和脑干受累是已被反复确认的pCMS危险因素,但它们无法解释其潜伏期。对于其他因素,如脑积水、术后脑膜炎、蚓部切口长度和肿瘤大小,研究结果不明确或为阴性。本研究的目的是确定围手术期的临床、影像学和实验室因素,这些因素也会增加pCMS发生的风险。重点关注可能解释pCMS延迟发作的因素,从而可能提供一个时间窗口来采取预防措施以预防pCMS或减轻其严重程度。该研究专门针对接受髓母细胞瘤手术的儿童。方法:在这项单中心回顾性队列研究中,作者纳入了71例髓母细胞瘤患儿,其中28例在初次切除后发生了pCMS。前瞻性收集临床和实验室数据并进行系统分析。纳入变量进行单因素和多因素分析。结果:单因素回归分析显示7个变量对pCMS发作有显著影响,即肿瘤大小、最大肿瘤直径>5 cm、肿瘤浸润或压迫脑干、pCMS组术后血红蛋白(p = 0.010)和血细胞比容(p = 0.003)的下降幅度明显大于无pCMS组、pCMS组手术期间肿瘤床严重出血事件报告更多、术前脑积水以及pCMS组术后前4天平均体温升高0.5°C。多因素回归分析显示肿瘤大小、肿瘤浸润或压迫脑干以及术后前4天较高的平均体温是pCMS的独立且高度显著的预测因素。结论:作者证实了早期的研究结果,即与肿瘤相关的术前情况,如最大肿瘤直径≥5 cm以及浸润或压迫脑干,与pCMS发生的较高风险相关。最重要的是,作者发现术后前4天平均体温升高0.5°C使pCMS发生的比值比增加近5倍。这些数据表明,对于接受髓母细胞瘤手术的儿童,预防pCMS的一个重要重点可能是术后严格维持正常体温作为标准护理措施。