Wang Chengjun, Zhao Meng, Deng Xiaofeng, Wang Jia, Shu Qingming, Jiang Zhongli, Zhao Jizong
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing, 100050, People's Republic of China.
China National Clinical Research Center for Neurological Diseases, Beijing, People's Republic of China.
Neurosurg Rev. 2018 Jul;41(3):799-811. doi: 10.1007/s10143-017-0930-0. Epub 2017 Dec 4.
Cavernous malformations (CMs) located at the foramen of Monro (FM) are relatively rare benign vascular malformations. Knowledge of FM CM is poor. The aims of this study were to describe the incidence, clinical presentation, radiological features, surgical approaches, and neurological outcomes for FM CM patients and to discuss the treatment strategy for this disease. We present a series of nine FM CM patients (four males, five females; mean age 29.3 years) who were treated at a single neurosurgical center. FM CM accounted for 0.56% of the entire series of the central nervous system (CNS) CMs. Headache accompanied by nausea and vomiting was the most common initial symptom (55.6%). The mean preoperative Karnofsky Performance Scale (KPS) score was 84.4 (range 70-100). In all but one patient, the lesions were surgically resected. Postoperatively, two patients developed obstructive hydrocephalus, and one experienced motor aphasia and right hemiparesis. At the time of discharge, the KPS score improved to a mean of 88.9. Follow-up period after diagnosis was 18 to 131 months (mean 69.7 months); all the patients were considered to be in excellent clinical condition. FM CMs are rare and challenging lesions; they have a female predilection. The most common clinical manifestations of FM CM are the symptoms of mass effect. The seizure risk of FM CMs seems to be significantly lower than that of general intraventricular CMs. Early surgical intervention should be offered to symptomatic cases, and gross total resection is associated with favorable neurological outcomes.
位于孟氏孔（FM）的海绵状血管畸形（CMs）是相对罕见的良性血管畸形。人们对FM CM的了解较少。本研究的目的是描述FM CM患者的发病率、临床表现、影像学特征、手术方法和神经学转归，并讨论该疾病的治疗策略。我们报告了在单一神经外科中心接受治疗的9例FM CM患者（4例男性，5例女性；平均年龄29.3岁）。FM CM占整个中枢神经系统（CNS）CM系列的0.56%。伴有恶心和呕吐的头痛是最常见的初始症状（55.6%）。术前卡氏功能状态评分（KPS）的平均得分为84.4（范围70 - 100）。除1例患者外，所有患者均接受了手术切除。术后，2例患者发生梗阻性脑积水，1例出现运动性失语和右侧偏瘫。出院时，KPS评分平均提高到88.9。诊断后的随访期为18至131个月（平均69.7个月）；所有患者的临床状况均被认为极佳。FM CMs是罕见且具有挑战性的病变；它们在女性中更为常见。FM CM最常见的临床表现是占位效应的症状。FM CMs的癫痫风险似乎明显低于一般的脑室内CMs。对于有症状的病例应尽早进行手术干预，全切除与良好的神经学转归相关。
