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儿童脑干表皮样囊肿:基于病例的最新进展

Epidermoid cyst of the brainstem in children: case-based update.

作者信息

Gopalakrishnan C V, Dhakoji Amit, Nair Suresh

机构信息

Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Trivandrum, India.

出版信息

J Child Neurol. 2012 Jan;27(1):105-12. doi: 10.1177/0883073811414709. Epub 2011 Aug 23.

Abstract

Epidermoid cysts are rare, slow growing, space-occupying lesions of early adulthood. Among the various locations, involvement of the brainstem is quite exceptional. In children, these tumors are extremely uncommon; only 4 purely intrinsic brainstem epidermoids are reported in the literature. The pathogenesis of true intraaxial brainstem epidermoid is unclear and is usually not considered in the preoperative differential diagnosis of brainstem tumors. We report 2 additional cases of brainstem epidermoid cysts occurring in children, underlining their clinical characteristics, the difficulties faced in the diagnostic work-up, and the surgical treatment adopted. These tumors pose a surgical challenge and attempts at aggressive removal of the cyst wall are fraught with increased risks of morbidity and mortality. On the other hand, cases treated more conservatively show good or excellent results with minimal complications. Neurosurgical judgment at the time of surgery is warranted to ensure maximum resection while minimizing postoperative neurologic deficits.

摘要

表皮样囊肿罕见,生长缓慢,是成年早期的占位性病变。在各个部位中,脑干受累相当罕见。在儿童中,这些肿瘤极为少见;文献中仅报道了4例单纯起源于脑干的表皮样囊肿。真正的轴内脑干表皮样囊肿的发病机制尚不清楚,在脑干肿瘤的术前鉴别诊断中通常不予以考虑。我们报告另外2例发生于儿童的脑干表皮样囊肿病例,强调其临床特征、诊断检查中面临的困难以及所采用的手术治疗方法。这些肿瘤带来了手术挑战,积极尝试切除囊肿壁会带来更高的发病和死亡风险。另一方面,采用更保守治疗的病例效果良好或极佳,并发症极少。手术时需要神经外科医生做出判断,以确保最大程度切除肿瘤,同时将术后神经功能缺损降至最低。

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