背景:小儿颅底病变很少发生,病因各异。传统上,开颅手术一直是首选的治疗方法;然而,如今,内镜方法的应用越来越多。在这个回顾性案例系列中,我们描述了我们治疗小儿颅底病变的经验,并对有关小儿颅底病变的治疗和结果的文献进行了系统的概述。
方法:我们对所有儿科患者(<18岁)进行了回顾性数据收集,巴塞尔大学儿童医院,瑞士,2015年至2021年。此外,还对现有文献进行了描述性统计和系统综述。
结果:我们纳入了17例患者,平均年龄8.92(±5.76)岁,9例男性(52.9%)。最常见的实体是鞍区病变(n=847.1%),其中颅咽管瘤是最常见的病理(n=4,23.5%)。内窥镜方法,经鼻蝶或经室,在9例(52.9%)中使用。6例患者(35.3%)发生了短暂性术后并发症,而在所有患者中,这些都不是永久性的。在9例(52.9%)术前缺陷患者中,2例(11.8%)在手术后完全康复,1例(5.9%)部分康复.在筛选了363篇文章后,我们纳入了16项研究,共807例患者进行系统评价.文献中报道的最常见的病理学证实了我们发现的颅咽管瘤(n=142,18.0%)。所有纳入研究的平均PFS为37.73(95%CI[36.2,39.2])个月,总体加权并发症发生率为40%(95%CI[0.28,0.53],永久性并发症发生率为15%(95%CI[0.08,0.27]).只有一项研究报告他们的队列5年总生存率为68%。
结论:本研究强调了儿童人群颅底病变的稀有性和异质性。虽然这些病理通常是良性的,由于病变的深层定位和雄辩的相邻结构,实现GTR具有挑战性,导致高并发症发生率。因此,儿童颅底病变需要经验丰富的多学科团队提供最佳护理.
BACKGROUND: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective
case series, we describe our experience in treating pediatric skull base lesions and provide a systematic overview of the literature on the treatment and outcome of pediatric skull base lesions.
METHODS: We conducted a retrospective data collection of all pediatric patients (<18 years) treated for a skull base lesion at the Division of Pediatric Neurosurgery, University Children\'s Hospital Basel, Switzerland, between 2015 and 2021. Descriptive statistics and a systematic review of the available literature were additionally conducted.
RESULTS: We included 17 patients with a mean age of 8.92 (±5.76) years and nine males (52.9%). The most common entity was sellar pathologies (n = 8 47.1%), with craniopharyngioma being the most common pathology (n = 4, 23.5%). Endoscopic approaches, either endonasal transsphenoidal or transventricular, were used in nine (52.9%) cases. Six patients (35.3%) suffered from transient postoperative complications, while in none of the patients these were permanent. Of the nine (52.9%) patients with preoperative deficits, two (11.8%) showed complete recovery and one (5.9%) partial recovery after surgery. After screening 363 articles, we included 16 studies with a total of 807 patients for the systematic review. The most common pathology reported in the literature confirmed our finding of craniopharyngioma (n = 142, 18.0%). The mean PFS amongst all the studies included was 37.73 (95% CI [36.2, 39.2]) months, and the overall weighted complication rate was 40% (95% CI [0.28 to 0.53] with a permanent complication rate of 15% (95% CI [0.08 to 0.27]. Only one study reported an overall survival of their cohort of 68% at five years.
CONCLUSIONS: This study highlights the rarity and heterogeneity of skull base lesions in the pediatric population. While these pathologies are often benign, achieving GTR is challenging due to the deep localization of the lesions and eloquent adjacent structures, leading to high complication rates. Therefore, skull base lesions in children require an experienced multidisciplinary team to provide optimal care.