Santos Maria M, Tannuri Ana Cristina A, Gibelli Nelson E, Ayoub Ali A, Maksoud-Filho João G, Andrade Wagner C, Velhote Manoel C P, Silva Marcos M, Pinho Maria L, Miyatani Helena T, Susuki Liza, Tannuri Uenis
Liver Transplantation Unit, Children's Institute, Hospital das Clinicas, University of Sao Paulo Medical School, Sao Paulo, Brazil.
Pediatr Transplant. 2011 Mar;15(2):157-60. doi: 10.1111/j.1399-3046.2010.01430.x. Epub 2010 Dec 13.
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.
后部可逆性脑病综合征(PRES)是一种神经临床和放射学综合征,由使用钙调神经磷酸酶抑制剂免疫抑制剂治疗引起。通常会出现严重高血压,但有些患者可能血压正常。我们在此报告两名接受肝移植的儿童,第一例是因胆道闭锁接受治疗,第二例是因阿拉吉耶综合征接受治疗。术后早期,两名患者均出现高血压和癫痫发作。在这两例中,影像学检查结果提示PRES诊断。CT扫描显示脑后部区域有改变,脑部MRI显示顶叶和枕叶区域有高信号强度。两名儿童均通过更换免疫抑制方案和控制动脉血压进行治疗。他们完全康复,没有任何神经后遗症。PRES的病理生理学可能是由于负责为脑后部区域供血的椎基底循环交感神经支配稀疏所致。总之,所有出现PRES神经症状的肝移植儿童都应在鉴别诊断中予以考虑,尽管这是一种罕见的并发症。作为治疗方法,我们建议严格控制动脉血压并更换免疫抑制方案。