Diagnosis and management of posterior reversible encephalopathy syndrome in pediatric transplant patients
Taner Sezer1, Emre Karakaya2, Ozan Okyay2, Adem Safak2, Sedat Yildirim2, Figen Ozcay3, Mehmet Haberal2.
1Department of Pediatric Neurology, Baskent University, Ankara, Turkey; 2Department of General Surgery, Division of Transplantation, Baskent University, Ankara, Turkey; 3Department of Pediatric Gastroenterology, Baskent University, Ankara, Turkey
Introduction: Posterior reversible encephalopathy syndrome (PRES) is a rare neurotoxic status composed of own brain imaging patterns. Depending on the location of the lesion, is characterized by a wide scale of neurologic symptoms. Although, the main cause of PRES is not fully enlightened, it is shown that, especially in solid organ transplant patients due to calcineurin inhibitors (CNI) toxicity, severe hypertension and any kind of infections are strongly related with PRES. The current therapy accepted is only supportive care. In this study we aim to evaluate diagnosis and management of PRES in our transplant patients.
Methods: From 3 November 1975 to 04 April 2025, we performed 3630 kidney transplant, 776 liver transplant and 154 heart transplants. Among these transplants 428 patient in kidney,377 patient in liver and 73 patients in heart were pediatric. In 12 pediatric transplant patients we observed PRES and median age was 12 years (5 month - 18 year). The radiology report database was analyzed for patients which PRES was cited in brain MR imaging text reports. Cases of PRES were included and searched for demographic values, laboratory values, clinical presentation, immunosuppressive they use, management we made and results.
Results: Among PRES cases, 6 (50%) patients were liver transplant, 4 (33%) were kidney transplant and 2 (17%) were heart transplant patients. %66,5 (n:8) of the patients were using Tacrolimus as immunosuppressant. Causes of PRES were toxicity of Tacrolimus in 3 (25%) patients, hypertension in 4 (33%) patients, sepsis in 2 (16%) patients, hypomagnesemia in 1(8,5%) patient, hyponatremia in 1 (8,5%) patient and cyclosporine toxicity in 1(8,5%) patient. Symptoms of PRES were Seizure in 8 (66,5%) patients, vision change in 3 (25%) patients, status epilepticus in 1 (8,5%) patient and nausea/ vomiting in 1 (8,5%) patient. The average time between transplant and the diagnosis of PRES was 23 days (2 - 27 days). In brain MRI edema (n:5), hemorrhagic lesions (n:4), ischemia (n:1) and brain infarction (n:1) were observed. Patients having hypertension (n:4) are treated with antihypertensives ACEİ combined with calcium canal blockers. Seizure is controlled with hydantoin at early period and levetiracetam (36.9 mg/kg/day) as maintenance treatment. Patients having hypertension are taken to hemodialysis. Also, patients related with tacrolimus toxicity (n:3) were switched to cyclosporine and the patient who had cyclosporine toxicity was switched to tacrolimus. End of the treatment period all patients were discharged successfully.
Conclusion: PRES is a rare diagnosis which is having an unknown etiology and due to that doesn’t have a proven management strategy. When the patient is suspected of PRES, MRI should be performed immediately, in order to avoid complications, achieve a better recovery and treat PRES successfully.