Application of shunt in pediatric diffuse intrinsic pontine glioma complicated with hydrocephalus: a 10-year experience from a single center

Abstract OBJECTIVE Currently, there is no unified treatment protocol for pediatric diffuse intrinsic pontine glioma with hydrocephalus. The present study aims to explore the clinical characteristics and the best intervention modality for pediatric diffuse intrinsic pontine glioma with hydrocephalus. METHODS The detailed data of children with diffuse intrinsic pontine glioma admitted to the Department of Pediatrics of Guangdong Sanjiu Brain Hospital from January 2010 to December 2021 were retrospectively analyzed. The effect of radiotherapy and shunt on the prognosis of pediatric diffuse intrinsic pontine glioma complicated with hydrocephalus was evaluated. RESULTS 165 children with DIPG aged ≤18 years were included in the analysis, of whom 9 (5.5%) had mild ventricular dilatation, 48 (35%) had hydrocephalus, and 11 (22.9%) underwent cerebrospinal fluid shunting, of whom 10 (90.9%) underwent ventriculoperitoneal shunt and 1 (9.1%) underwent endoscopic third ventriculostomy. After shunt and radiotherapy, 53 cases of hydrocephalus could be evaluated, of which 37 patients (69.8%) were significantly relieved, and 16 (30.2%) were not. 99 children had tumor progression after treatment, including 41 cases (41.4%) with hydrocephalus and 5 cases (12.2%) with ventriculoperitoneal shunt. A total of 138 children were included in the survival analysis, which revealed that hydrocephalus, mild ventricular dilatation, and no ventricular abnormality had no effect on the prognosis (p=0.944). The treatment of hydrocephalus at the initial diagnosis did not affect the prognosis, p=0.405. The median survival of the hydrocephalus remission group post-radiotherapy was 12 months, and 8 months for the non-remission group, p=0.042. The median survival was 20 months in the shunt group and 8 months in the untreated group (p=0.005). CONCLUSIONS Asymptomatic ventricular enlargement in pediatric diffuse intrinsic pontine gliomas does not require surgical intervention. Hydrocephalus remission post-radiotherapy is a favorable prognostic indicator. Cerebrospinal fluid shunt could improve the prognosis of children with hydrocephalus when the tumor progresses post-radiotherapy.