ID #790 Posterior Reversible Encephalopathy Syndrome in Pediatric Brain Tumor Patients – A Distinct Entity
Mallory Owen, Jennifer Yang, Maria Montenegro, Helen Harvey, Nicole Coufal, Michael Levy, John CrawfordAbstract
Background
PRES is a potentially severe condition with abrupt onset of neurologic symptoms, often precipitated by uncontrolled hypertension. PRES in pediatric primary brain tumor (PBT) is rare and its unique clinical and radiologic characteristics are underreported.
Methods
We conducted a single center retrospective cohort study of all patients with PRES under 18 years from January 1, 2000 to January 1, 2025, paired with a comprehensive literature review for cases of PRES in PBT. We compared clinical and radiologic features of PBT-PRES and non-PBT PRES cases using descriptive analyses, Fisher’s Exact Test for categorical variables and Wilcoxon rank-sum test for continuous variables. A p-value <0.05 was considered significant.
Results
We included 19 PBT-PRES cases (5 from our institution, 14 from literature review, median age 9 years, IQR=6.5-12.5, 79% male) and 47 non-PBT PRES cases from the single-center cohort (median age 11 years, IQR=8.5-13.5, 34% male). In PBT-PRES, 78% had posterior fossa tumors; 71% developed PRES within 30 days of intracranial surgery. PBT PRES had a higher proportion of males than the non-PBT group (79% vs 34%, p < 0.001), ongoing neurologic sequelae (50% vs 4%, p < 0.001), severity necessitating intubation (75% vs 30%, p = 0.021), and 90-day mortality (17% vs 0%, p = 0.019). Radiologically, PBT-PRES had increased brainstem involvement (28% vs 4%, p = 0.015), hemorrhagic conversion (60% vs 11%, p = 0.002), diffusion restriction (50% vs 15%, p = 0.026), and contrast enhancement (50% vs 14%, p = 0.035). There were no significant differences in highest systolic blood pressure, length of admission, ICU admission, or supratentorial imaging pattern.
Conclusions
PBT-PRES is a rare entity associated with unique clinical/radiographic features and high morbidity/mortality compared to non PBT-PRES. The perioperative PBT-PRES findings may represent an expanded phenotype of posterior fossa syndrome or post-operative hydrocephalus.