Treatment of hydrocephalus following fetal repair of myelomeningocele: comparing endoscopic third ventriculostomy with choroid plexus cauterization to ventricular shunting.

Abstract

The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC). 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). 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. 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.