Posterior reversible encephalopathy syndrome after kidney transplantation in pediatric recipients: Two cases

Abstract

PRES is a neuro-clinical and radiological syndrome that can result as a consequence of several different conditions including hypertension, fluid overload, and immunosuppressive treatment. Herein, we report two children who received kidney and combined liver-kidney transplantation as treatment for renal hypodysplasia associated with bilateral vesico-ureteral reflux and methylmalonic acidemia, respectively. Early after surgery (seven and 10 days), both patients presented with hypertension and seizures. The patients' immunosuppressive regimen included steroid and calcineurin inhibitors (tacrolimus and cyclosporine, respectively) and basiliximab and one with anti-IL2 receptor. In both cases, the imaging strongly supported the diagnosis of PRES. In details, the CT scan showed hypodensities in the posterior areas of the brain, and brain MRI demonstrated parieto-occipital alterations indicative of vasogenic edema. Treatment with calcineurin inhibitors was temporally discontinued and restarted at lower dosage; arterial hypertension was treated with Ca-channel blockers. Both children fully recovered without any neurological sequels. In conclusion, in children undergoing solid organ transplantation, who develop neurological symptoms PRES, should be carefully considered in the differential diagnosis and once the diagnosis is ruled in, we recommend strict arterial blood pressure control and adjustment or withholding of calcineurin inhibitor therapy should be considered based upon blood levels.

Posterior Reversible Encephalopathy Syndrome in Kidney Disease

“… 28 Another possible strategy is to withdraw the presumed offending drug at the time of PRES and then re-introduce it at a lower dose once PRES has resolved. 29 Opinions vary on the subject, and the lack of a definitive strategy reflects a paucity of studies on the condition.…”

Meta-analysis of cognitive functioning in patients following kidney transplantation

“…However, longitudinal follow-up studies included in this review suggest that symptoms of PRES are resolved in the long term compared with pre-transplant levels [ 14 , 24 , 46 ]. Investigations of PRES have been explored, but only on a case study [ 76 ] or small sample size basis [ 77 ]. Therefore, the role and identification of PRES warrants further investigation in longitudinal designs.…”

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

“…14,47,50,52,101,104,105,115,122,125,155 Commonly identified etiologic precipitants include renal disease, hypertension, electrolyte disturbances, infection, connective tissue and autoimmune disease, thrombotic microangiopathy, transplantation, immunosuppressive therapy, malignancy, and chemotherapy. 32,35,38,43 In pediatric patients, PRES most commonly occurs in the context of hypertension, renal disease, leukemia, chemotherapy, and HSCT. 7 Clinical manifestations variably include headache of moderate to severe intensity, decreased level of consciousness, visual-field and acuity disturbances, and epileptic activity.…”