Encapsulating peritoneal sclerosis in children

Abstract

Encapsulating peritoneal sclerosis (EPS) is a rare but extremely serious complication of peritoneal dialysis (PD). While EPS has been well recognized in adults on long-term PD, and children can spend many years on PD before a transplant becomes available, only a small number of children with EPS have been described. Two European pediatric registries have recently reported on the prevalence, potential risk factors and outcomes of EPS in children. Although the prevalence of EPS is comparable to that published in adult registries, the outcome of pediatric EPS is significantly better and carries a lower mortality. All studies have shown a greater risk of EPS with a longer dialysis vintage, but it is not known why some individuals are susceptible to EPS development. In this review we discuss current views on the epidemiology, pathogenesis and management strategies for EPS. The hope of the authors is that this review will alert pediatric nephrologists to this rare but extremely serious complication of chronic PD. In the future, collaborative research and the establishment of a pediatric EPS registry may be of importance in helping pediatric nephrologists to recognize the early warning signs of EPS development and thereby to develop strategies for its prevention and optimal management.