摘要目的:分析囊壁上附着异位脉络丛的鞍上池囊肿的临床特点和治疗方法，并探讨囊肿增大的机制。方法:回顾性分析2017年2月至2022年2月上海交通大学医学院附属上海儿童医学中心神经外科收治的19例鞍上池囊肿患儿的临床资料。患儿的中位年龄为9.4个月(1~48个月)。17例在母亲孕检中发现鞍上池囊肿并行胎儿MRI明确诊断，另2例分别在出生后6、42个月发现并确诊。术前MRI显示，17例患儿的囊肿呈进行性增大。19例患儿中，2例囊肿仍局限于鞍上池生长，另17例自鞍区向周边扩大并压迫颞叶、顶叶、枕叶、小脑或脑干。2例伴中度脑积水，7例脑室受压变小，另10例脑室大小在正常范围内。8例在神经内镜下经颞部行囊肿－脑池开窗术，11例在神经内镜下经额叶行囊肿－第三脑室－桥前池开窗术。术后定期门诊随访，进行影像学复查以评估囊肿的变化及有无复发。结果:术中发现，19例患儿的鞍区部位囊壁上均有散在的珊瑚状脉络丛结构。所有患儿均顺利完成手术，无手术并发症发生。19例患儿均获得随访，随访时间为(12.8±7.0)个月(3~24个月)，17例患儿生长发育良好，且囊肿缩小>50%，MRI检查显示囊肿无复发；2例行囊肿－脑池开窗术的患儿出现术后复发，分别接受囊肿－腹腔分流术和囊肿－第三脑室－桥前池开窗术后好转。结论:胎儿及儿童早期发现的鞍上池囊肿具有快速增大且向周边扩展的临床特点，其囊壁上的异位脉络丛主动分泌脑脊液可能是囊肿增大的主要机制，神经内镜下囊肿－第三脑室－桥前池开窗术应为首选治疗方法。

abstractsObjective:To investigate the clinical characteristics and treatment of suprasellar arachnoid cysts (SAC) with ectopic choroidal heterotopia and to explore the mechanisms of SAC enlargement.Methods:The clinical data of 19 cases with SAC admitted to the Department of Neurosurgery, Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from February 2017 to February 2022 were retrospectively studied. The patient′s median age was 9.4 months (1-48 months). SAC was identified in 17 cases by fetus MRI when the mothers underwent physical examination during pregnancy and in the other 2 cases in early childhood (6 and 42 months respectively) and then the diagnosis of SAC were established. Progressive enlargement of SAC was shown in 17 patients before operation. The cyst was localized in the suprasellar cistern in 2 cases, while the cyst in the remaining 15 cases expanded from the suprasellar cistern to the periphery, compressing the temporal lobe, parietal lobe, occipital lobe, cerebellum or medulla oblongata. Two cases were accompanied with moderate hydrocephalus. The ventricles shrank and shifted due to compression by cysts in 7 cases. Normal ventricles were found in 10 cases. Endoscopic cystocisternostomy (ECC) was performed in 8 patients and endoscopic third ventriculocystocisternostomy (EVCC) was performed in 11. Regular imaging follow-up was used to observe the changes of the cysts to determine whether the cysts recurred.Results:The choroidal heterotopia in suprasellar arachnoid cyst inside the sellar region was identified in all cases during operations. The procedures were successfully performed without any complications. The mean clinical follow-up period was 12.8±7.0 months(3-24 months)in 19 children. The size of the cyst decreases by >50% in 17 patients with good growth and development and MRI showed no recurrence. Two patient who underwent ECC had lesion recurrence post operation. Of them, recurred cyst shrank after cystoperitoneal shunting in 1 case and resolved after EVCC in the other.Conclusions:The SAC identified in fetus and the early stage of childhood shows the clinical characteristics of rapid enlargement and expansion to adjacent regions. Secretory action of the ectopic choroidal heterotopia may play an important role in the mechanism of SAC. EVCC should be the primary treatment method for suprasellar cistern cyst.