PRES in Children Undergoing Hematopoietic Stem Cell or Solid Organ Transplantation.
Posterior reversible encephalopathy syndrome (PRES) is a clinical neuroradiologic entity that is becoming increasingly well known and documented in pediatrics. It is characterized by a variable association of seizures, headache, vomiting, altered mental status, visual disturbances, and seizures, as well as imaging suggesting white-gray matter edema involving the posterior regions of the central nervous system in most cases. The pathophysiology of PRES remains unclear. Although PRES has been associated with a widespread range of clinical conditions, namely infections, adverse drug events, autoimmune diseases, and many others, its onset after hematopoietic stem cell and solid organ transplantation remains the most commonly reported. Historically, PRES has proved to be generally reversible and associated with good clinical outcomes; however, severe complications, sometimes life-threatening, can also occur. Most reported cases of childhood PRES after hematopoietic stem cell or solid organ transplantation have been case reports or series across a broad spectrum of different transplant settings, and no clear consensus exists regarding how best to manage the syndrome. Thus, in this article, we provide a comprehensive review of the pathophysiological, clinical, and diagnostic aspects of PRES in children, with a specific focus on the transplant scenario. Differential diagnoses with other neurologic complications after pediatric transplantation are reviewed, and crucial issues in the management of PRES and the development of future research are ultimately addressed.
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, University of Bologna, Bologna, Italy; and.
Riccardo
R
Masetti
Department of Pediatric Neurology, Sant'Orsola Malpighi Hospital, Bologna, Italy ducciomaria.cordelli@aosp.bo.it.
Duccio Maria
DM
Cordelli
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, University of Bologna, Bologna, Italy; and.
Daniele
D
Zama
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, University of Bologna, Bologna, Italy; and.
Francesca
F
Vendemini
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, University of Bologna, Bologna, Italy; and.
Carlotta
C
Biagi
Department of Pediatric Neurology, Sant'Orsola Malpighi Hospital, Bologna, Italy.
Emilio
E
Franzoni
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, University of Bologna, Bologna, Italy; and.
Andrea
A
Pession
D002648:Child; D003663:Decision Trees; D003937:Diagnosis, Differential; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016377:Organ Transplantation; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis / Q000628:therapy; D011183:Postoperative Complications / Q000175:diagnosis / Q000628:therapy
PRES; children; hematopoietic stem cell transplantation; posterior reversible encephalopathy syndrome; solid organ transplantation