Nagib M G, O'Fallon M T
Neurosurgical Associates, Ltd., Minneapolis, MN, USA.
Pediatr Neurosurg. 1996 Jun;24(6):299-305. doi: 10.1159/000121059.
Over a period extending from 1984 to 1993, 16 children ranging in ages from 2 months to 12 years with posterior fossa ependymoma were treated. Four of these tumors were classified as lateral ependymomas because of their configuration and suspected site of origin. These 4 patients' ages ranged from 2 months to 5 years. Signs and symptoms of increased intracranial pressure and cranial nerve dysfunction were the hallmark of their presentation. Their imaging evaluation included a preoperative and postoperative CT san and/or MRI. A gross total surgical resection' was completed in the 4 children. At least a 1-year follow-up was available for all the patients. Intraoperative brain stem evoked potentials and facial and glossopharyngeal nerve monitoring were used in all the surgeries. Three children required a ventriculoperitoneal shunt postoperatively. Chemotherapy was used postoperatively in 3 children. The 4th child did not receive chemotherapy due to parental refusal and succumbed to a recurrence 1 years postoperatively. Radiation therapy was given to 2 children. Tumor recurrences developed in all children at 12 months, 18 months, 3 years and 4 years postoperatively in proximity to the original tumor site. Histologic sections in 3 patients, including those at the time of recurrence, were consistent with a low mitotic index. Only 1 child's histologic diagnosis was consistent with a high-grade ependymoma. The lower cranial nerve dysfunction transiently worsened in all the patients and was permanent in 2. The child's age, tumor histology and extent of resection play an important role in the prognosis of posterior fossa ependymoma. The lateral posterior fossa ependymoma has a particularly poor prognosis due to its location and its significant postoperative morbidity. The authors propose a combined midline and lateral suboccipital approach in order to expose the fourth ventricular floor, upper cervical spine, lateral recess, cerebellomedullary fissure, cerebellopontine and the cerebellomedullary cistern in an attempt at a "total' resection with a focus on the site of origin. The usage of neurophysiological monitoring appears to be useful in limiting and predicting the extent of postoperative complications. However, prolonged morbidity is likely and is commonly related to lower cranial nerve deficits. The author proposes a proactive approach in order to limit the sequelae of these complications.
在1984年至1993年期间，对16名年龄在2个月至12岁的后颅窝室管膜瘤患儿进行了治疗。其中4例肿瘤因其形态和可疑起源部位被归类为外侧室管膜瘤。这4例患者的年龄在2个月至5岁之间。颅内压升高和颅神经功能障碍的体征和症状是其主要表现。他们的影像学评估包括术前和术后的CT扫描和/或MRI。4名儿童均完成了肿瘤全切除。所有患者均至少有1年的随访。所有手术均使用了术中脑干诱发电位以及面神经和舌咽神经监测。3名儿童术后需要进行脑室腹腔分流术。3名儿童术后接受了化疗。第4名儿童因家长拒绝未接受化疗，术后1年死于复发。2名儿童接受了放射治疗。所有儿童在术后12个月、18个月、3年和4年时在原肿瘤部位附近均出现了肿瘤复发。3例患者（包括复发时的患者）的组织学切片显示有丝分裂指数较低。只有1名儿童的组织学诊断与高级别室管膜瘤一致。所有患者的低位颅神经功能障碍均有短暂恶化，2例为永久性恶化。患儿的年龄、肿瘤组织学类型和切除范围在后颅窝室管膜瘤的预后中起着重要作用。后颅窝外侧室管膜瘤因其位置和显著的术后发病率，预后特别差。作者提出采用联合中线和枕下外侧入路，以暴露第四脑室底、上颈椎、外侧隐窝、小脑延髓裂、小脑脑桥角和小脑延髓池，试图进行“全”切除，重点关注起源部位。神经生理监测的使用似乎有助于限制和预测术后并发症的程度。然而，长期发病率很可能出现，且通常与低位颅神经缺损有关。作者提出一种积极的方法以限制这些并发症的后遗症。
