ESPN 51th Annual Meeting

ESPN 2018


 
Posterior Reversible Encephalopathy Syndrome in two peritoneal-dialyzed children
TAMARA KELLLER 1 DAVID-ALEXANDER WILLE 2 DANIELA MARX-BERGER 1 GUIDO F. LAUBE 1

1- PEDIATRIC NEPHROLOGY UNIT, UNIVERSITY CHILDRENS HOSPITAL ZURICH, SWITZERLAND
2- PEDIATRIC NEUROLOGY UNIT, UNIVERSITY CHILDRENS HOSPITAL ZURICH, SWITZERLAND
 
Introduction:

Posterior Reversible Encephalopathy Syndrome (PRES) is a syndrome characterized by seizures, focal neurological signs, altered mental status, headache and visual disorders with typical, reversible radiographic findings. Underlying conditions are arterial hypertension, renal diseases (e.g. uremia, nephrotic syndrome, HUS, renal transplantation) and calcineurin inhibitors. We present two children on peritonealdialysis (PD) with PRES assuming additive pathophysiological aspects.

Material and methods:

case report

Results:

Patient 1: Girl with ARPKD bilaterally nephrectomised at 6 months because of massive kidney size leading to uncontrollable arterial hypertension and failure to thrive. At 8 months, untreated blood pressure became low (70/40 mmHg) and she presented with altered mental state, focal seizures and a blood pressure of 90/50 mmHg at admission. Laboratory findings were uneventful (creatinine 375 ┬Ámol/l; urea 12 mmol/l). MRI revealed symmetric T2-hyperintensity and diffusion restriction in both occipital lobes. PD was withhold 1 day. Anticonvulsive treatment was initially administered and tapered thereafter. The patient fully recovered within 4 months. Patient 2: Six-year-old girl with Shigatoxin positive HUS on PD (maximal blood pressure 134/100 mmHg) indicated visual impairment leading to total blindness within 2 hours followed by a non-convulsive status epilepticus. MRI revealed T2-hyperintensity and diffusion restriction located in both occipital lobes. Antihypertensive treatment was optimized. Two days later, vision improved. PD could be stopped and antihypertensive treatment was tapered. Two weeks later the girl was dismissed with total recovery.

Conclusions:

We present PRES not caused by high, but also low blood pressure indicating general blood pressure fluctuations as a possible trigger. Relevant fluid shifts on PD might lead to altering blood pressure. Additionally endothelial cell dysfunction might explain PRES, as activation and malfunction of the immune system (e.g. HUS and persistent uremia) results in chronic, subclinical inflammation. The net result is endothelial activation and damage, followed by increased endothelial permeability causing brain edema and therefore PRES.