3岁儿童症状性动眼神经囊肿1例,并强调手术治疗

M. Müther, Ann-Katrin Bruns, T. Fortmann, A. Brentrup, U. Grenzebach, W. Stummer
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摘要

小儿第三神经麻痹最常见的原因是创伤、肿瘤或血管异常。然而,囊性动眼神经病变是罕见的。我们报告一个有症状的囊肿沿着和在动眼神经,这是以前没有描述的情况。在这里,我们报告一个3岁的女孩表现为进行性无痛动眼神经麻痹。磁共振成像显示沿神经池状和海绵状方向有囊性形成。由于缺乏其他治疗方案,只能进行手术。术中直接神经刺激可识别囊肿壁的无功能部分,并进行开窗和活检。组织病理学显示为神经炎。多种病原菌血清学检测均为阴性。动眼性麻痹迅速消失。术后随访5年,女孩无症状,囊肿的蓄水池部分仍然塌陷。这是首个通过开窗减压有效治疗沿动眼神经及内的症状性囊肿的报道,强调了颅神经手术中术中神经监测的重要性。这种疾病的病因仍不确定。
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Symptomatic Oculomotor Nerve Cyst in a 3-Year-Old Child: Case Report With Emphasis on Surgical Management
Third nerve palsies in the pediatric population are most commonly caused by trauma, tumors, or vascular abnormalities. Cystic oculomotor nerve neuropathies, however, are rare. We report the case of a symptomatic cyst along and within the oculomotor nerve, which has not been described previously. Here, we report a case of a 3-yr-old girl presenting with a progressive painless oculomotor nerve palsy. A magnetic resonance imaging revealed a cystic formation along the cisternal and cavernous course of the nerve. Due to lack of alternative treatment options, surgery was offered. Intraoperative direct nerve stimulation allowed for identification of a non-functional part of the cyst wall and open fenestration and biopsy were executed. Histopathology revealed neuritis. Serology was negative for various pathogens. The oculomotor palsy rapidly resolved. At a follow-up 5 yr after surgery, the girl is asymptomatic and the cisternal part of the cyst remains collapsed. This is the first report of a symptomatic cyst along and within the oculomotor nerve treated effectively with open fenestration and decompression highlighting the importance of intraoperative neuromonitoring in cranial nerve surgery. Uncertainty remains regarding the etiology of this disease.
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