Sudden Blindness after Enterocystoplasty – A Case Report

Introduction

Posterior reversible encephalopathy syndrome (PRES) has first been described by Hinchey in 1996. He based his observations on neuroradiological findings of edema prevailing in the parieto-occipital regions of the cerebral hemispheres, caused by altered functional vascularization (Hinchey et al. N Engl J Med 1996; 334: 494−500). It mainly occurs as an acute disorder in patients with immunosuppressive therapy, chronic kidney disease (CKD) or arterial hypertension and preeclampsia. Symptoms vary from headaches to seizures, altered mental status as well as visual disturbances (Bartynski WS. AJNR Am J Neuroradiol 2008; 29: 1036−1042).

In recent years, PRES has been described also amongst children with kidney disorders like acute glomerulonephritis, lupus nephritis, nephrotic syndrome and renal transplant recipients. Two theories regarding etiology have been proposed: First, severe hypertension leads to failed auto-regulation, subsequent hyperperfusion, with endothelial injury/vasogenic edema. Second, vasoconstriction and hypoperfusion lead to brain ischemia and subsequent vasogenic edema (Yamada A, Ueda N. Pediatr Nephrol 2012; 27: 277−283).

Publication History

Article published online:
04 January 2021