HYDROCEPHALUS TREATMENT AND THE EFFECT ON SURVIVAL IN DIFFUSE INTRINSIC PONTINE GLIOMA

Abstract BACKGROUND: Diffuse intrinsic pontine glioma (DIPG), can cause hydrocephalus and if symptomatic, leads to rapid changes in consciousness requiring surgical intervention. The effect of cerebrospinal fluid (CSF) diversion on overall survival and the clinical factors influencing outcome remain unclear. The aim of this study was to evaluate the impact of the treatment of hydrocephalus on survival in DIPG patients. METHODS: The study was retrospective in design using data from the SIOPE-European Society of Pediatric Oncology DIPG Registry. Hydrocephalus was determined based on a centrally reviewed diagnostic MRI. The Kaplan-Meier method was used for survival statistics. Clinical prognostic factors including: duration of symptoms, age and cranial nerve palsy at diagnosis were evaluated for confounding and effect modification. The effect of hydrocephalus treatment (CSF diversion) on survival was examined using Cox regression. RESULTS: Among 582 patients from the SIOPE-DIPG Registry, 86 (14%) had hydrocephalus at diagnosis. Median OS for hydrocephalus patients treated with CSF diversion (n=43) was 13 months (95% CI, 10.2-17.7) and 9 months (95% CI, 7.4-10.6) for hydrocephalus patients without a CSF diversion (n=43). Survival rates were not significantly different (p=.41). On adjusted Cox regression, correcting for duration of symptoms, hydrocephalus patients with signs of cranial nerve palsy at diagnosis and a CSF diversion had a hazard ratio 0.476 (p=0.004). CONCLUSION: Survival among DIPG patients presenting with hydrocephalus at diagnosis was not influenced by CSF diversion. Hydrocephalus patients with signs of cranial nerve palsy at diagnosis, had a significantly reduced risk after undergoing CSF diversion. There is an indication this subgroup of DIPG patients may benefit more from CSF diversion, although the relationship between hydrocephalus and cranial nerve palsy requires further investigation.