Fonseca Adriana, Solano Palma, Ramaswamy Vijay, Tabori Uri, Huang Annie, Drake James M, Tsang Derek S, Laperriere Normand, Bartels Ute, Kulkarni Abhaya V, Bouffet Eric
1Division of Haematology Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
2Division of Oncology, Department of Pediatrics, Hospital Virgen Del Rocio, Seville, Spain.
J Neurosurg. 2021 Mar 5;135(4):1139-1145. doi: 10.3171/2020.10.JNS203257. Print 2021 Oct 1.
There is no consensus on the optimal clinical management of ventriculomegaly and hydrocephalus in patients with diffuse intrinsic pontine glioma (DIPG). To date, the impact on survival in patients with ventriculomegaly and CSF diversion for hydrocephalus in this population remains to be elucidated. Herein, the authors describe their institutional experience.
Patients diagnosed with DIPG and treated with up-front radiation therapy (RT) at The Hospital for Sick Children between 2000 and 2019 were identified. Images at diagnosis and progression were used to determine the frontal/occipital horn ratio (FOR) as a method to measure ventricular size. Patients with ventriculomegaly (FOR ≥ 0.36) were stratified according to the presence of symptoms and categorized as follows: 1) asymptomatic ventriculomegaly and 2) symptomatic hydrocephalus. For patients with ventriculomegaly who did not require CSF diversion, post-RT imaging was also evaluated to assess changes in the FOR after RT. Proportional hazards analyses were used to identify clinical and treatment factors correlated with survival. The Kaplan-Meier method was used to perform survival estimates, and the log-rank method was used to identify survival differences between groups.
Eighty-two patients met the inclusion criteria. At diagnosis, 28% (n = 23) of patients presented with ventriculomegaly, including 8 patients who had symptomatic hydrocephalus and underwent CSF diversion. A ventriculoperitoneal shunt was placed in the majority of patients (6/8). Fifteen asymptomatic patients were managed without CSF diversion. Six patients had resolution of ventriculomegaly after RT. Of 66 patients with imaging at the time of progression, 36 (55%) had ventriculomegaly, and 9 of them required CSF diversion. The presence of ventriculomegaly at diagnosis did not correlate with survival on univariate analysis. However, patients with symptomatic hydrocephalus at the time of progression who underwent CSF diversion had a survival advantage (p = 0.0340) when compared to patients with ventriculomegaly managed with conservative approaches.
Although ventriculomegaly can be present in up to 55% of patients with DIPG, the majority of patients present with asymptomatic ventriculomegaly and do not require surgical interventions. In some cases ventriculomegaly improved after medical management with steroids and RT. CSF diversion for hydrocephalus at the time of diagnosis does not impact survival. In contrast, our results suggest a survival advantage in patients who undergo CSF diversion for hydrocephalus at the time of progression, albeit that advantage is likely to be confounded by biological and individual patient factors. Further research in this area is needed to understand the best timing and type of interventions in this population.
对于弥漫性脑桥内生型胶质瘤（DIPG）患者脑室扩大和脑积水的最佳临床管理尚无共识。迄今为止，该人群中脑室扩大和脑积水行脑脊液分流对生存的影响仍有待阐明。在此，作者描述了他们机构的经验。
确定2000年至2019年期间在病童医院被诊断为DIPG并接受 upfront 放射治疗（RT）的患者。诊断和病情进展时的图像用于确定额/枕角比（FOR），作为测量脑室大小的方法。脑室扩大（FOR≥0.36）的患者根据症状的有无进行分层，并分类如下：1）无症状性脑室扩大和2）症状性脑积水。对于不需要脑脊液分流的脑室扩大患者，RT后的影像学检查也用于评估RT后脑室扩大的变化。采用比例风险分析来确定与生存相关的临床和治疗因素。采用Kaplan-Meier方法进行生存估计，采用对数秩检验方法确定组间生存差异。
82例患者符合纳入标准。诊断时，28%（n = 23）的患者出现脑室扩大，其中8例有症状性脑积水并接受了脑脊液分流。大多数患者（6/8）置入了脑室腹腔分流管。15例无症状患者未进行脑脊液分流进行处理。6例患者RT后脑室扩大消退。在66例病情进展时有影像学检查的患者中，36例（55%）有脑室扩大，其中9例需要脑脊液分流。诊断时脑室扩大的存在在单因素分析中与生存无关。然而，与采用保守方法处理脑室扩大的患者相比，病情进展时出现症状性脑积水并接受脑脊液分流的患者具有生存优势（p = 0.0340）。
尽管高达55%的DIPG患者可出现脑室扩大，但大多数患者表现为无症状性脑室扩大，不需要手术干预。在某些情况下，使用类固醇和RT进行药物治疗后脑室扩大有所改善。诊断时因脑积水进行脑脊液分流不影响生存。相比之下，我们的结果表明，病情进展时因脑积水接受脑脊液分流的患者具有生存优势，尽管这种优势可能会受到生物学和个体患者因素的混淆。该领域需要进一步研究以了解该人群中最佳的干预时机和类型。
