Hussen Endris, Aboye Samson, Leake Merhawi, Nuredin Abrar Fadil
Department of Neurosurgery, St. Paul's Millennium Medical College, Addis Ababa, Ethiopia.
Minilik II Comprehensive Specialized Hospital, Addis Ababa, Ethiopia.
Int Med Case Rep J. 2023 Sep 12;16:521-527. doi: 10.2147/IMCRJ.S430853. eCollection 2023.
Central nervous system tumors are usually located in the brain, and spinal cord tumors account for approximately 20% of central nervous system tumors. Epidermoid cysts constitute <1% of all intraspinal tumors. It consists of squamous epithelial-lined cysts containing keratin, cholesterol, and cellular granules. Epidermoid cysts can be classified as congenital, acquired, extradural, extramedullary, or intramedullary according to etiology and location. The intradural intramedullary type is uncommon.
An 11-year-old female patient had back pain with radiation to both lower extremities but worsened on the left side for 5 years. On neurological examination, motor strength was 3/5 for hip flexion and knee extension bilaterally and 5/5 for other key muscle groups. Contrast-enhanced lumbosacral magnetic resonance imaging (MRI) revealed T1 hypointense and T2 hyperintense lesions in the L2-L4 intramedullary conus and cauda equina. Laminectomy and near total resection were done, and histopathological examination revealed an epidermoid cyst. On the third postoperative day, the patient was discharged with completely resolved back pain and an improvement in lower extremity motor power. At monthly follow-up visits for a further 6 months, her back pain and weakness completely resolved, and she had no neurologic deficits. A postoperative lumbosacral MRI was done and confirmed near total excision of the tumor.
Intramedullary conus epidermoid cysts are rare but not unknown to neurosurgeons. MRI with diffusion-weighted images (DWI) is an imaging modality of choice. Asymptomatic patients can be conservatively treated. Once the patient has progressive symptoms and signs of compression, surgical excision is recommended. Meticulous electrocauterization may help decrease tumor regrowth in the remnant capsule, which is recommended. Avoiding leakage of cyst fluid into the subarachnoid space helps to avoid postoperative chemical meningitis. Radiotherapy is an option for the management of multiple recurrences.
中枢神经系统肿瘤通常位于脑部，脊髓肿瘤约占中枢神经系统肿瘤的20%。表皮样囊肿占所有脊髓内肿瘤的比例不到1%。它由含有角蛋白、胆固醇和细胞颗粒的鳞状上皮内衬囊肿组成。表皮样囊肿可根据病因和位置分为先天性、后天性、硬膜外、髓外或髓内。髓内硬膜内型并不常见。
一名11岁女性患者背痛伴双下肢放射性疼痛，但左侧疼痛加重5年。神经系统检查显示，双侧髋关节屈曲和膝关节伸展的肌力为3/5，其他关键肌肉群的肌力为5/5。增强型腰骶部磁共振成像（MRI）显示L2-L4髓内圆锥和马尾神经区域T1低信号和T2高信号病变。进行了椎板切除术和近全切术，组织病理学检查显示为表皮样囊肿。术后第三天，患者出院，背痛完全缓解，下肢运动能力有所改善。在接下来的6个月每月随访中，她的背痛和无力完全缓解，且无神经功能缺损。术后进行了腰骶部MRI检查，证实肿瘤近全切。
髓内圆锥表皮样囊肿罕见，但神经外科医生对此并不陌生。带有扩散加权成像（DWI）的MRI是首选的成像方式。无症状患者可进行保守治疗。一旦患者出现进行性症状和压迫体征，建议手术切除。细致的电灼术可能有助于减少残余囊内肿瘤的复发，推荐采用。避免囊肿液漏入蛛网膜下腔有助于避免术后化学性脑膜炎。放射治疗是处理多次复发的一种选择。
