Preoperative prediction of postoperative cerebellar mutism syndrome. Validation of existing MRI models and proposal of the new Rotterdam pCMS prediction model

Abstract

Purpose Postoperative cerebellar mutism syndrome (pCMS) is a complication that may occur after pediatric fossa posterior tumor surgery. Liu et al. developed an MRI-based prediction model to estimate pCMS risk preoperatively. The goal of this study was to validate the model of Liu et al. and if validation was not as sensitive in our group as previously described to develop an easy to use, reliable, and sensitive preoperative risk prediction model for pCMS. Methods In this study, 121children with a fossa posterior tumor who underwent surgery at ErasmusMC/Sophia Children's Hospital, the Netherlands between 2004 and 2018 could be included. Twenty-six percent of them developed pCMS. Preoperative MRI were scored using the Liu et al. model. Results The Liu et al. model reached an accuracy of 78%, a sensitivity of 58%, and a specificity of 84% in our cohort. In a new risk model some of the variables of Liu et al. were included as well as some of the recently described preoperative MRI characteristics in pCMS patients by Zhang et al. The new model reached an accuracy of 87%, a sensitivity of 97%, and a specificity of 84% in our patient group. Conclusion Because the Liu et al. model did not provide an as accurate risk prediction in our cohort as was expected, we created a new risk prediction model that reached high model accuracy in our cohort that could assist neurosurgeons in determining their surgical tactics and help prepare high risk patients and their parents for this severe complication.

Mapping of long-term cognitive and motor deficits in pediatric cerebellar brain tumor survivors into a cerebellar white matter atlas

“…As gross total tumor resection is critical for lowering event-free survival in both patient groups an approach to safeguarding motor and cognitive function may lead to conflicting aims in the management of these patients. A solution could be using radiologic algorithms of conventional MRI as well as fMRI and DTI that may identify preoperatively patients at risk of developing postoperative cerebellar mutism [42,43] which is the most predictive complication for long-term deficits [44]. Advanced MRI radiomics [45] and liquid biopsy from cerebrospinal fluid [46] may further enhance preoperative diagnostic accuracy.…”

Cerebellar mutism syndrome: From pathophysiology to rehabilitation

“…In recent years, several models of preoperative risk stratification have been investigated, using both imaging and clinical features (Liu et al, 2018;Zhang et al, 2019;Bae et al, 2020). The estimation of a high risk of mutism following a complete resection has implications not only to the extent that this must be carefully discussed by the neurosurgical team but also for the surgical strategy itself.…”

“…In addition to having a potential impact on the assessment process and surgical strategy, a risk score for the development of post-operative CMS in the future could also be useful in selecting patients for preventive neuroprotective therapies once these have been developed (Toescu et al, 2018b;Liu et al, 2018;Bae et al, 2020).…”

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options

“…Finally, intra-operative tissue damage to the region of the vermis is reported to share an intimate relationship with persisting affective disturbances as part of the ''cerebellar cognitive affective syndrome'' [56]. Apart from that, a lot of researchers have developed imaging-based predictive models in order to elucidate the underlying pathophysiology of CMS [26,57]. The DTC pathway represents an important outflow tract from the cerebellar nuclei towards the cerebral cortex, as it connects the dentate nucleus, via the contralateral red nucleus and thalamus, to the contralateral cerebral cortex There is consensus that interruption of this pathway constitutes the main pathological substrate for CMS [8,58].…”