Doherty Tara M, Gruffi Catherine, Overby Philip
Pediatric Anesthesiology, Westchester Medical Center, Valhalla, USA.
Pediatric Neurology, Westchester Medical Center, Valhalla, USA.
Cureus. 2021 Nov 9;13(11):e19414. doi: 10.7759/cureus.19414. eCollection 2021 Nov.
Propofol infusion syndrome was first reported in the literature by Bray in 1998. He described a series of fatal outcomes after a presenting constellation of symptoms observed in pediatric patients who had received prolonged propofol infusions. Profound metabolic acidosis and bradycardia are the disease's hallmark features, which can further develop expeditiously to rhabdomyolysis, renal failure, and heart failure. It has been subsequently theorized that a triggering mechanism or a precipitating factor sets up the progressive physiologic spiral which can ensue. The name of the disease was expanded to Propofol Related Infusion Syndrome (PRIS), as propofol alone was no longer considered the culprit. The disease process is rare and can present with an insidious onset in some cases, causing much speculation of whether there is a proper grasp of the disease entity in its entirety as currently reported. The case discussed in this article depicts an adverse neurologic outcome following a craniotomy for temporal lobectomy in a child with lesional epilepsy. Since there was no obvious causative factor for these findings, PRIS became a diagnosis that was robustly discussed among the involved services.
丙泊酚输注综合征于1998年由布雷首次在文献中报道。他描述了在接受长时间丙泊酚输注的儿科患者中观察到的一系列症状出现后的致命结局。严重代谢性酸中毒和心动过缓是该疾病的标志性特征，可迅速进一步发展为横纹肌溶解、肾衰竭和心力衰竭。随后有人提出，一种触发机制或诱发因素引发了可能随之而来的渐进性生理螺旋式变化。该疾病的名称扩展为丙泊酚相关输注综合征（PRIS），因为不再认为仅丙泊酚是罪魁祸首。该疾病过程罕见，在某些情况下可能隐匿起病，引发了许多关于目前报道中是否对该疾病实体有全面正确认识的猜测。本文讨论的病例描述了一名患有病灶性癫痫的儿童在颞叶切除开颅术后出现的不良神经学结局。由于这些发现没有明显的病因，PRIS成为相关科室深入讨论的诊断。
