Department of Pediatric Neurosurgery, Great Ormond Street Hospital for Children, London , UK.
Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto, Ontario, Canada.
Neurosurgery. 2024 Oct 1;95(4):859-876. doi: 10.1227/neu.0000000000002948. Epub 2024 Apr 29.
Anterior basal encephaloceles are considered a rare entity and are often associated with midline cerebral abnormalities. Those with a large skull base defect and herniation of brain parenchyma in the neonate or young infant present unique challenges for surgical management.
We analyzed the neurosurgical administrative and operative databases between 1986 and 2022 to determine clinical presentation, operative approach, and outcome of basal encephaloceles.
Over the 36-year period, 27 pediatric anterior basal encephaloceles were managed, of which 22 had full documentation and images allowing comprehensive review. Mean age at presentation was 5 years (SD 4.94). The majority were transethmoidal encephaloceles (59%), followed by the transsphenoidal-sphenoethmoidal type (32%). Overall, 91% were managed surgically by a transcranial, endoscopic, or combined approach. Four children required subsequent procedures, predominantly for persistent cerebrospinal fluid leak. No significant differences in proportion of patients requiring interval/revision surgery after initial conservative, endoscopic endonasal, or transcranial surgery was identified. Neither age at surgery nor size of the defect on computed tomography scan was associated with the need for revision surgery. Size of cranial defect was significantly smaller in the endoscopic group ( P = .01). There was a historic tendency for younger children with larger defects to have a transcranial approach. With the addition of endoscopic skull base expertise, smaller defects in older children were more recently treated endoscopically.
Basal encephaloceles are rare and complex lesions and are optimally managed within a skull base multidisciplinary team able to provide multiple approaches. Large skull base defects with brain parenchymal involvement often require a transcranial or combined transcranial-endoscopic approach.
前颅底脑膨出被认为是一种罕见的疾病，常与中线脑异常相关。对于新生儿或婴幼儿期颅底骨质缺损较大、脑组织疝出的患者，手术治疗具有独特的挑战性。
我们分析了 1986 年至 2022 年期间的神经外科行政和手术数据库，以确定基底脑膨出的临床表现、手术入路和结局。
在 36 年期间，共管理了 27 例儿童前颅底脑膨出，其中 22 例有完整的记录和图像可供全面评估。患者的平均年龄为 5 岁（标准差为 4.94）。大多数为经筛窦脑膨出（59%），其次为经蝶窦-蝶筛窦型（32%）。总体而言，91%的患者通过经颅、内镜或联合入路进行手术治疗。4 例患儿需要进一步手术，主要是为了治疗持续性脑脊液漏。首次保守治疗、内镜经鼻或经颅手术后，需要间隔/再次手术的患者比例无显著差异。手术年龄或 CT 扫描上的缺损大小与再次手术的需要无关。内镜组的颅缺损大小明显较小（P=0.01）。既往存在较大缺损的儿童行经颅入路的倾向较大，而随着内镜颅底专业知识的增加，近期对于较大缺损的年长儿童，更倾向于采用内镜入路。
基底脑膨出是一种罕见且复杂的病变，最好在能够提供多种入路的颅底多学科团队中进行管理。伴有脑实质受累的大颅底骨质缺损通常需要经颅或联合经颅-内镜入路治疗。
