Different Clinicoradiological Characteristics of Posterior Reversible Encephalopathy Syndrome in Pediatric Oncology and Post-Bone Marrow Transplantation Cases: A Retrospective Study

Abstract

Posterior reversible encephalopathy syndrome (PRES) is receiving increasing recognition in pediatrics. However, comparisons between PRES in pediatric oncology and post-bone marrow transplantation (BMT) are lacking. Therefore, we aimed to describe the risk factors and clinical and radiological features of PRES and investigate the differences between PRES in pediatric oncology and post-BMT. The PRES data of 13 patients from our center were combined with those of 217 cases from the PubMed, Scopus, and Web of Science databases. The patients were divided into either an oncology or a post-BMT group. We included 230 patients in the analysis, 26.1% of whom belonged to the post-BMT group. Oncology patients developed PRES at a younger age (p = 0.010) and were more likely to develop encephalopathy (p = 0.004). Systemic hypertension (S-HTN) preceding PRES occurred in 43.5% (66/154) of patients. Post-BMT patients were more likely to have S-HTN (p = 0.003). Cyclosporine levels were detected in 37 patients; 40.5% had supra-therapeutic levels. The radiological findings were atypical in 74.3% of patients, and delayed repeated imaging increased the occurrence of resolution (p = 0.004). Sixteen (7%) patients developed PRES recurrence after a median of 8 weeks, with the between-group difference being non-significant. Oncology patients were more likely to develop chronic epilepsy, while BMT patients were more likely to develop rare neurologic abnormalities (p < 0.001). In conclusion, atypical clinical presentation and imaging findings should not hinder the diagnosis of PRES. S-HTN is a risk factor, particularly in post-BMT patients. Supra-therapeutic levels of cyclosporine and previous exposure to immunosuppression did not increase the risk of recurrence.

Posterior reversible encephalopathy syndrome post stem cell transplantation in sickle cell disease: case series and literature review

“…In children with hemoglobinopathies, PRES has been reported in patients treated with calcineurin inhibitors (cyclosporin, sirolimus and tacrolimus) ( 4 , 6 , 19 , 20 ). In one series of children with SCD and thalassemia who underwent SCT from Rome (age range 2–17 years old), PRES was reported in 31 of 222 cases.…”

“…PRES has been reported with several hematological disorders including thalassemia and sickle cell disease (SCD) ( 4–6 ). In SCD, most cases have been reported in children in close proximity to stem cell transplantation (SCT).…”

Review of acute kidney injury and progression to chronic kidney disease in pediatric patients undergoing hematopoietic cell transplant

“…Shah et al. studied the difference between PRES in pediatric oncology and post-HCT patients and found that oncology patients developed PRES at a younger age and were more likely to present with encephalopathy ( 70 ). Systemic HTN preceded PRES in 43.5% of patients, and this was more likely in post-HCT patients.…”

“…The primary composite endpoint of 50% GFR decline and ESKD favored the intensive BP arm (HR: 0.65; 95% CI: 0.44-0.94) (69). Children with systemic HTN have a narrower range of autoregulation in cerebral blood flow and an increased risk of cerebrovascular dysfunction, making them more susceptible to posterior reversible encephalopathy syndrome (PRES) (70). PRES presents with an abrupt, acute rise in BP along with seizures, visual changes, encephalopathy, headache, and radiologic findings on brain magnetic resonance imaging (MRI) with focal reversible vasogenic edema (71).…”

“…PRES is a neurological emergency requiring early identification and management including gradual reduction in BP and removal of any identifiable offending agents (72). Shah et al studied the difference between PRES in pediatric oncology and post-HCT patients and found that oncology patients developed PRES at a younger age and were more likely to present with encephalopathy (70). Systemic HTN preceded PRES in 43.5% of patients, and this was more likely in post-HCT patients.…”