Posterior reversible encephalopathy syndrome after liver transplantation in children: A rare complication related to calcineurin inhibitor effects

Abstract

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.

Early and late neurological complications of liver transplantation in pediatric patients

“…PRES is a clinical/radiologic entity involving posterior lesions in the cerebral hemispheres characterized by alterations in consciousness, cortical blindness, and convulsions . The incidence of PRES in pediatric LT recipients was reported to be 0.8%‐10% and due primarily to toxicities of calcineurin inhibitors . In our study, the incidence of PRES was 2.9% and all of the PRES attacks in three patients were characterized by radiologic changes, either typical or atypical for PRES (Figure ).…”

Liver transplantation during infancy: No increased rate of neurological complications

“…The reported incidence of PRES after pLT ranges from 0.8% to 10% . Indeed, PRES was previously associated to CsA therapy, which was the standard immunosuppressive drug in our collective as well. The absence of PRES in our patients may derive from the specific age structure of our cohort.…”

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

“…Since its first description in early 1990, many cases of PRES in organ transplantation recipients have been described, but the majority of them are adults. The few reports involving children seem to indicate a higher frequency of PRES in this age group (as high as >5%) (9)(10)(11)(12)(13). In a study reporting neurological complications occurring after renal transplantation in children, two cases of acute postoperative PRES over 113 operated children (2.2%) have been observed (10).…”