Intrinsic brainstem epidermoid cyst in childhood.

Surgical neurology international Pub Date : 2025-07-18 eCollection Date: 2025-01-01 DOI:10.25259/SNI_198_2025
Tybault Hollanders, Sarah Hendrickx, Edward Baert
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Abstract

Background: Epidermoid cysts are slow-growing, rare congenital lesions. They are most seen in the cerebellopontine angle, fourth ventricle, or parasellar regions. Patients typically become symptomatic between 20- and 40 years of age. We present a rare case of a symptomatic intrinsic brainstem epidermoid cyst in a 12-year-old girl with atypical radiological features.

Case description: A 12-year-old girl presented with progressive gait disturbances, disequilibrium, diplopia due to right-sided abducens paresis, left-sided facial paresis (HB grade II), headaches, nausea, and vomiting over the past 6 months. Magnetic resonance imaging (MRI) revealed an intrinsic lesion of the brainstem not present on MRI 4 years prior. A microsurgical gross total resection, including resection of cyst wall, was performed. The pathology report diagnosed the lesion as an epidermoid cyst. The patient improved significantly after resection.

Conclusion: This pathology represents <1% of all intracranial tumors, and with only 21 documented pediatric intrinsic brainstem cases in medical literature, very rarely has an intrinsic brainstem location. We detail the medical history, work-up, surgical management, and postoperative outcomes, contributing to the limited body of knowledge regarding this exceptional entity.

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儿童内源性脑干表皮样囊肿。
背景:表皮样囊肿是一种生长缓慢、罕见的先天性病变。最常见于桥小脑角、第四脑室或鞍旁区。患者通常在20至40岁之间出现症状。我们报告一例罕见的有症状的内源性脑干表皮样囊肿,发生于一名12岁的女孩,影像学表现不典型。病例描述:一名12岁女孩,在过去的6个月里表现为进行性步态障碍,不平衡,右侧外展肌轻瘫,左侧面部轻瘫(HB II级)导致的复视,头痛,恶心和呕吐。磁共振成像(MRI)显示脑干固有病变,4年前MRI未见。显微外科大体全切除,包括囊肿壁切除。病理报告诊断为表皮样囊肿。术后患者病情明显好转。结论:该病理表现为
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