LAUSR

Abstract BACKGROUND The is no consensus in best practices for the management of hydrocephalus in patients with Diffuse Intrinsic Pontine Glioma (DIPG). To date, the impact on survival of hydrocephalus and Cerebro-Spinal Fluid (CSF) diversion in this population remains to be elucidated. Herein, we describe our institutional experience. METHODS Patients with a clinical and radiological diagnosis of DIPG were identified at the Hospital for Sick Children between 2000–2019. Images at diagnosis and at disease progression were assessed for hydrocephalus using the frontal-occipital ratio (FOR) method. Proportional hazard analyses were used to identify factors correlated with survival. RESULTS Eighty-nine consecutive patients diagnosed with DIPGs were treated at our institution. At diagnosis, 29% (n=26) of patients presented with hydrocephalus, seven patients underwent CSF diversion. Out of the remaining nineteen patients, n=6 had stable or improved hydrocephalus in follow-up scans, n=6 had persistent hydro and n=2 required CSF diversion at the time of disease progression. Seven did not undergo a follow-up scan. Out of sixty-five patients with imaging at the time of progression, fifty-five percent of patients (n=36) presented with hydrocephalus and ten of them required CSF diversion. On univariate analysis, the presence of hydrocephalus or CSF diversion at diagnosis and/or did not correlate with a survival advantage. CONCLUSIONS CSF diversion for the management of hydrocephalus in patients with DIPG does not impact survival and in some cases resolves spontaneously after the initiation of radiotherapy and steroids. This observation needs to be validated in a prospective cohort.