Long-term postoperative quality of life in childhood survivors with cerebellar mutism syndrome

Abstract

Background: To investigate the long-term quality of life (QoL) of children with cerebellar mutism syndrome (CMS) and explore the risk factors for a low QoL. Procedure: This cross-sectional study investigated children who underwent posterior fossa surgery using an online Pediatric Quality of Life Inventory questionnaire. CMS and non-CMS patients were included to identify QoL predictors. Results: Sixty-nine patients were included (male, 62.3%), 22 of whom had CMS. The mean follow-up time was 45.2 months. Children with CMS had a significantly lower mean QoL score (65.3 vs. 83.7, p < 0.001) and subdomain mean scores (physical; 57.8 vs. 85.3, p < 0.001; social: 69.5 vs. 85.1, p = 0.001; academic: p = 0.001) than those without CMS, except for the emotional domain (78.0 vs. 83.7, p = 0.062). Multivariable analysis revealed that CMS (coefficient = −14.748.61, p = 0.043), chemotherapy (coefficient = −7.629.82, p = 0.013), ventriculoperitoneal (VP) shunt placement (coefficient = −10.14, p = 0.024), and older age at surgery (coefficient = −1.1830, p = 0.007) were independent predictors of low total QoL scores. Physical scores were independently associated with CMS (coefficient = −27.4815.31, p = 0.005), VP shunt placement (coefficient = −12.86, p = 0.025), and radiotherapy (coefficient = −13.62, p = 0.007). Emotional score was negatively associated with age at surgery (coefficient = −1.92, p = 0.0337) and chemotherapy (coefficient = −9.11, p = 0.003). Social scores were negatively associated with male sex (coefficient = −13.68, p = 0.001) and VP shunt placement (coefficient = −1.36, p = 0.005), whereas academic scores were negatively correlated with chemotherapy (coefficient = −17.45, p < 0.001) and age at surgery (coefficient = −1.92, p = 0.002). Extent of resection (coefficient = 13.16, p = 0.021) was a good predictor of higher academic scores. Conclusion: CMS results in long-term neurological and neuropsychological deficits, negatively affecting QoL, and warranting early rehabilitation.