Dorfer Christian, Ochi Ayako, Snead O Carter, Donner Elizabeth, Holowka Stephanie, Widjaja Elysa, Rutka James T
Division of Pediatric Neurosurgery, The Hospital for Sick Children, Suite 1503, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada.
Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
Childs Nerv Syst. 2015 Nov;31(11):2103-9. doi: 10.1007/s00381-015-2794-3. Epub 2015 Jun 23.
We report on our experience in performing peri-insular functional hemispherectomy (PIH) in very young infants with catastrophic epilepsy.
We retrospectively reviewed the medical charts of all infants with catastrophic epilepsy that underwent PIH under the age of 4 months at our institution.
Four infants (three female, one male) were included (median age at time of surgery 2.9 months, range from 2.4 to 4.2 months; median patient's weight at time of surgery 5650 g, range from 4300 to 7500 g). None of the patients experienced hemodynamic instability during surgery. All four patients were given red blood cell replacement (median 435 ml, range from 230 to 800 ml), three of the four patients experienced coagulopathy during surgery and were given platelet cells transfusion in one (50 ml) and fresh frozen plasma in two patients (191 and 320 ml). Two patients experienced severe complications that, however, did not cause a permanent morbidity due to prompt diagnosis and correct management. After a median follow-up time of 4.3 years (range from 1.3 to 7.9 years), three of four patients are completely seizure free. The remaining patient is experiencing brief daily staring episodes. All of them have a hemiparesis but are fully ambulatory and have a useful upper limb function.
In catastrophic epilepsy, PIH within the first months of life is feasible provided that an experienced multidisciplinary team is involved. Awareness of surgical challenges and potential complications is indispensible when the life-threatening nature of the epilepsy compels neurosurgeons to operate at this very young age.
我们报告在患有灾难性癫痫的非常年幼婴儿中进行岛周功能性大脑半球切除术(PIH)的经验。
我们回顾性分析了在我们机构接受PIH手术的所有4个月以下患有灾难性癫痫婴儿的病历。
纳入4例婴儿(3例女性,1例男性)(手术时的中位年龄为2.9个月,范围为2.4至4.2个月;手术时患者的中位体重为5650克,范围为4300至7500克)。所有患者在手术期间均未出现血流动力学不稳定。所有4例患者均接受了红细胞置换(中位值435毫升,范围为230至800毫升),4例患者中有3例在手术期间出现凝血功能障碍,其中1例接受了血小板输注(50毫升),2例患者接受了新鲜冰冻血浆输注(191和320毫升)。2例患者出现严重并发症,但由于诊断及时和处理得当,未导致永久性残疾。中位随访时间为4.3年(范围为1.3至7.9年)后,4例患者中有3例完全无癫痫发作。其余患者每天有短暂的凝视发作。他们均有偏瘫,但均可独立行走,上肢功能良好。
在灾难性癫痫中,若有经验丰富的多学科团队参与,在生命的头几个月内进行PIH是可行的。当癫痫的危及生命性质迫使神经外科医生在这个非常年幼的年龄进行手术时,意识到手术挑战和潜在并发症是必不可少的。