颅内血管畸形的神经眼科表现分析

Clinical analysis of neurophthalmological features in patients with intracranial vascular malformation

背景颅内血管畸形可因畸形血管破裂出血或本身占位性病变压迫视路而表现出不同的神经眼科改变，但因该病发病率相对较低，目前国内外仅有该病神经眼科表现的个案报道，尚缺乏系统临床研究。目的了解颅内血管畸形患者的神经眼科表现。方法系统性回顾分析2007年6月至2013年6月在中山大学附属第三医院收治的100例颅内血管畸形患者的一般情况、首发症状、神经眼科症状及体征、临床影像学检查资料等。结果100例颅内血管畸形患者中出现神经眼科表现者20例，占20％。20例伴有神经眼科改变的患者中，仅1例以视物模糊为首发症状，其余均以神经系统症状为首发而就诊。主要的神经眼科表现为视野缺损，占50％；视力下降占45％；瞳孔异常占35％。其他表现包括视神经萎缩1例，占5％；视盘水肿1例，占5％；动眼神经麻痹1例，占5％。CT和MRI检查发现，有眼科症状的20例患者中脑动静脉畸形者12例，占60％，病灶分别位于枕叶4例、顶枕叶3例、颞叶3例、额叶1例和中脑1例；海绵状血管瘤患者7例，占35％，病灶分别位于顶叶、枕叶、扣带回、海绵窦、颞叶、顶颞叶和桥脑，各1例；小脑静脉瘤合并海绵状血管瘤1例，占5％。20例患者中14例伴颅内出血，占70％，出血部位分别位于蛛网膜下腔6例和脑出血8例。结论颅内血管畸形患者可有不同的神经眼科表现，多由畸形血管破裂出血引起，应引起眼科医师重视。

Background Intracranial vascular malformation causes different neurophthalmological symptoms and signs due to oppressing visual-related tissue and cranial nerves after hemorrhage. However,there is no availably systematic clinical research on this disease up to now. Objective This retrospective cases analysis was to explore the neurophthalmological features of intracranial vascular malformation. Methods The clinical datas were collected from 100 cases with intracranial vascular malformation in Affiliated Third Hospital of Sun Yat-sen University from June,2007 to June, 2013. The neurophthalmological clinical features including general condition, initial symptom, concomitant symptoms and image results were retrospectively surveyed. Results Neurophthalmo]ogical symptoms and signs were found in 20 patients with intracranial vascular malformation by CT and MRI with the detectability 20% （20/100）. The main neurophthalmological symptoms were visual field defect （50% , 10/20） and vision loss （45% , 9/20）. The neurophthalmological signs were pupil abnormalities （35% ,7/20）. Other neurophthalmologieal features included optic nerve atrophy （ 5 % , 1/20 ） , papilloedema （ 5 % , 1/20 ） and oculomotor nerve palsy （ 5 % , 1/20） , etc. The relevant cranial diseases included cerebral arteriovenous malformation in 60% patients （ 12/20 ） , cavernous angiomas in 35% （7/20） and venous malformation with cavernous angiomas （1/20）. The lesions of cerebral arteriovenous malformation were located in occipital lobe （4 cases） , parietal-occipital area （3 cases） , temporal lobe （3 cases） and frontal lobe （1 case）,midbrain area （1 case）; while those of cavernous angiomas were located in parietal lobe （ 1 case） ,occipital lobe （ 1 case） , gyrus cingulated area occipital lobe （ 1 case） , carvenous sinus （ 1 case） ,temporal lobe （1 case）, parietal-temporal area （1 case） and pons area （1 case）. Intraeranial hemorrhageoccurred in 14 patients in subarachnoid space （6 cases） and brain （8 cases）. Conclusions The patients with intracranial vascular malformation have different neurophthalmological symptoms and signs. Sufficient attention should be paid to the patients with neurophthalmological symptoms and signs.