背景:室管膜囊肿(EC)通常存在于侧脑室附近,近室,或颞顶区域。以前的病例还发现了脑干的幕下EC,小脑,和蛛网膜下腔。他们大多是无症状的。在本文中,我们介绍了有史以来第一例手术治疗的有症状的髓质室管膜囊肿,以及关于脑干其他部位EC的文献的全面回顾。
方法:这位51岁的女性出现听力损失,头晕,复视,和共济失调.射线照相成像表明髓质中存在非增强性病变,对脑干有质量影响。病理检查证实其表征为室管膜囊肿。该患者在神经导航下接受了枕下开颅手术,以开窗治疗髓质室管膜囊肿,术中超声和术中神经监测。组织病理学检查证实了室管膜囊肿的诊断。在一个月的随访中,KPS为90,ECOGPS为1,共济失调得到改善,复视完全消退.
结论:由于它们的稀有性和与其他囊性结构的潜在相似性,EC可能被忽视或错误诊断,导致管理不善和手术灾难。因此,全面了解和认识其独特特征对于准确诊断和适当管理至关重要。
BACKGROUND: Ependymal cysts (EC) typically present supra-tentorially near the lateral ventricle, juxta ventricular, or temporoparietal regions. Previous cases have also identified infratentorial EC of the brainstem, cerebellum, and subarachnoid spaces. They are mostly asymptomatic. In this paper, we present the first-ever
case of a symptomatic medullary ependymal cyst treated with surgery, along with a comprehensive review of the literature on EC of other parts of the brain stem.
METHODS: This 51-year-old female presented with hearing loss, dizziness, diplopia, and ataxia. Radiographic imaging indicated the presence of a non-enhancing lesion in the medulla with a mass effect on the brainstem. Pathological examination confirmed its characterization as an ependymal cyst. The patient underwent a suboccipital craniotomy for the fenestration of the medullary ependymal cyst under neuro-navigation, Intra-op ultrasound and intra-operative neuro-monitoring. Histopathological examination confirmed the diagnosis of an ependymal cyst. At one month follow-up, her KPS is 90, ECOG PS 1, and her ataxia has improved with complete resolution of diplopia.
CONCLUSIONS: Due to their rarity and potential similarity to other cystic structures, EC may be overlooked or incorrectly diagnosed resulting in mismanagement and surgical disaster. Therefore, a comprehensive understanding and awareness of their distinct characteristics are essential for accurate diagnosis and appropriate management.