title: Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects.
abstract: PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.
authors.affiliation: Liver Transplantation Unit, Children's Institute, Hospital das Clinicas, University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Maria M
authors.initials: MM
authors.lastname: Santos
authors.forename: Ana Cristina A
authors.initials: AC
authors.lastname: Tannuri
authors.forename: Nelson E
authors.initials: NE
authors.lastname: Gibelli
authors.forename: Ali A
authors.initials: AA
authors.lastname: Ayoub
authors.forename: João G
authors.initials: JG
authors.lastname: Maksoud-Filho
authors.forename: Wagner C
authors.initials: WC
authors.lastname: Andrade
authors.forename: Manoel C P
authors.initials: MC
authors.lastname: Velhote
authors.forename: Marcos M
authors.initials: MM
authors.lastname: Silva
authors.forename: Maria L
authors.initials: ML
authors.lastname: Pinho
authors.forename: Helena T
authors.initials: HT
authors.lastname: Miyatani
authors.forename: Liza
authors.initials: L
authors.lastname: Susuki
authors.forename: Uenis
authors.initials: U
authors.lastname: Tannuri
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome; D001656:Biliary Atresia / Q000175:diagnosis / Q000601:surgery; D019703:Calcineurin / Q000009:adverse effects*; D065095:Calcineurin Inhibitors; D002648:Child; D003524:Cyclosporins / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D020343:Hypertensive Encephalopathy / Q000139:chemically induced / Q000175:diagnosis; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D016031:Liver Transplantation / Q000009:adverse effects* / Q000379:methods; D019520:Living Donors; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D054038:Posterior Leukoencephalopathy Syndrome / Q000139:chemically induced* / Q000175:diagnosis; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D014181:Transplantation Immunology / Q000502:physiology
chemical_list: D065095:Calcineurin Inhibitors; D003524:Cyclosporins; D007166:Immunosuppressive Agents; D019703:Calcineurin