眼眶Ⅰ型神经纤维瘤颅眶骨异常的CT征  

Cranio-Orbital Abnormalities in Orbital Neurofibromatosis Type Ⅰ

作　　者：高文[1] 朱利民[1] 何彦津[1] 林婷婷[1] 张薇[1] 孙世振 

机构地区：[1]天津医科大学眼科中心,天津市300070

出　　处：《中国实用眼科杂志》2010年第10期1099-1101,共3页Chinese Journal of Practical Ophthalmology

摘　　要：目的 探讨眼眶Ⅰ型神经纤维瘤（NF-1）颅眶骨异常的CT表现及机制.方法 2008年1月至2010年1月收治的7例眼眶NF-1患者中5例存在颅眶骨改变,分析该5例患者的临床资料和CT表现,全部病例均经手术病理和（或）临床证实.结果 5例眼眶NF-1中3例为颅眶沟通肿瘤（其中1例伴有上睑额部肿瘤）,2例为上睑、颞、额部肿瘤.全部存在不同程度的颅眶骨异常,主要表现：蝶骨大翼缺失5例,眶上裂增大5例,眶壁骨质缺失1例,蝶鞍变形1例,颞骨变形1例.另外,4例患者相应部位蛛网膜囊肿.结论 眼眶NF-1大部分存在不同程度的颅眶骨异常,以蝶骨大翼缺失最为显著.其发生机制复杂,可能同时存在先天发育异常和继发性改变,有待临床研究.Objective To review the possible manifestation and mechanism of Cranio-Orbital Changes in Orbital Neurofibromatosis Type Ⅰ （NF-1）. Methods The CT imaging abnormalities of the orbit and cranium in 5 patients with Orbital NF-1 were reviewed retrospectively. Results Orbital and cranial abnormalities were documented in 5 patients including 3 cases of tumor in cranium-orbit and 2 cases of tumor in upper eyelid, tempora, and forehead. Five cases had defect of the greater sphenoid wings; 5 had expansion of the orbital foramen, the superior orbital fissure; 1 had epippium abnormality, 1 had temporal bone abnormality,1 had defect of wall of orbit. In addition, 4 cases of orbital and cranial abnormalities contiguous to the arachnoid cyst. Conclusions Patients with orbit NF-1, bony orbital and cranial changes occur frequently, and defects of the greater sphenoid wings can be explained by a congenital neuroectodermal and mesodermal maldevelopment hypothesis. In addition, the orbital NF-1 mass and arachnoid cyst is associated with orbital and cranial abnormalities.

关 键 词：眼眶Ⅰ型神经纤维瘤 颅眶骨改变 CT 

分 类 号：R730.264[医药卫生—肿瘤]