METHODS: This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings).
RESULTS: Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors\' institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (-0.06 vs -0.09, p = 0.37) and change in head circumference percentile (-1.33 vs -28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort.
CONCLUSIONS: In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.
方法:这是一项回顾性队列研究,对象是2012年至2021年在范德比尔特通过VPS或ETV伴或不伴CPC(ETV±CPC)的fMMR后接受脑积水治疗的儿童。主要结果是治疗失败和失败时间(TTF)。次要结果包括脑积水指标的变化(额枕角比[FOHR]和头围测量)和医疗资源利用率(住院人数,诊所就诊,和神经影像学发现)。
结果:在88例接受fMMR的患者中,37(42%)需要永久分流脑脊液,其中19人在提交人的机构接受了治疗。12名患者接受了ETV±CPC,7例患者接受了VPS安置,校正年龄中位数为23周对1周(p=0.002).ETV±CPC队列患者的术前中位头围百分位数和z评分与VPS队列相似(百分位数:98.5vs94.0,p=0.064;z评分:2.32vs1.60,p=0.111)。两个队列之间的术前中位FOHR测量值没有差异(0.57vs0.59,p=0.53)。术后6个月,ETV±CPC队列的中位头围百分位数和z评分在两个队列之间保持相似(百分位数:98.0vs67.5,p=0.315;z评分:2.12vs0.52,p=0.307).在指数CSF转移程序后6个月,两组之间的FOHR变化(-0.06vs-0.09,p=0.37)和头围百分位数变化(-1.33vs-28.6,p=0.058)没有差异。ETV±CPC队列中的一名患者术后出现癫痫发作和非手术性硬膜下出血;未观察到其他并发症。VPS队列中的7例患者中有6例需要分流翻修,TTF中位数为9.8个月,而12例ETV±CPC患者中有2例需要重复ETV,中位数为17.5个月(86%vs17%,p=0.013)。ETV±CPC队列中脑积水相关医院再入院的中位数明显低于VPS队列(0vs1,p=0.006)。ETV±CPC队列的CT扫描(0vs2,p=0.004)和X光片(0vs2,p<0.001)少于VPS队列。
结论:在单中心队列中,通过ETV±CPC治疗的脑积水fMMR患者仍然没有分流,而大多数接受前期分流的患者需要翻修。这是第一项在fMMR脑积水人群中比较ETV±CPC与VPS的研究。虽然更大,需要多中心研究,这些结果表明,ETV/CPC可能是fMMR后CSF转移的首选手段。