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Published on: September 6, 2017
Posterior reversible encephalopathy syndrome in children after hematopoietic stem cell and solid organ
Noémie de Cacqueray1, Charles-Joris Roux2, Raphaël Levy2
1Pediatric intensive care unit, Hôpital Necker-Enfants Malades, APHP, Paris, France; Université Paris Cité, Inserm, Pharmacologie et évaluations des thérapeutiques chez l'enfant et la femme enceinte, F-75006 Paris, France.
Background:
Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome characterized by clinical and radiologic features that can occur in children under specific circumstances. Solid Organ Transplantation (SOT) and Hematopoietic Stem Cell Transplantation (HSCT) are the most commonly reported underlying conditions in children, although they have been studied separately and in small series.
Objective:
This study aimed to compare the characteristics of PRES in children following HSCT and SOT.
Methods And Settings:
We conducted a retrospective single-center study of children diagnosed with PRES after transplantation between 2007 and 2019 based on a review of local transplant databases. Neuroradiologists reviewed all brain magnetic resonance imaging (MRI) to confirm diagnoses. Clinical presentation, imaging findings, risk factors, and outcomes were compared between children after HSCT and SOT.
Results:
Among 548 HSCT and 556 SOT recipients, 33 PRES cases were initially suspected. After imaging review, 12 cases were excluded. Finally, nine cases of PRES following HSCT (median age 7.6 years, 6 males) and 12 following SOT (liver, kidney, and heart transplants, median age 8.9 years, 7 males) were included. PRES was a rare complication, occurring in 2% of patients undergoing HSCT and SOT (p= 0.66). Clinical presentation was similar in both groups, mainly with seizures (n=20, 95%). Hypertension and transplantation-related complications promoting endothelial damage were common in both groups. MRI showed typical posterior brain lesions in all patients, but also abnormal anterior regions in 19 (91%) patients and positive diffusion restriction in ten (48%). PRES was a severe complication, with frequent intensive care unit (ICU) admission (n=13, 62%).
Conclusion:
PRES is a rare but severe complication in children after transplantation, with a higher incidence and severity than previously reported. Our findings suggest a key role of endothelial dysfunction. Early recognition of risk factors and careful interpretation of MRI are crucial for timely diagnosis and management.
