CAJ | 학술논문

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.
목적 탐토안광Ⅰ형신경섬유류(NF-1)로광골이상적CT표현급궤제.방법 2008년1월지2010년1월수치적7례안광NF-1환자중5례존재로광골개변,분석해5례환자적림상자료화CT표현,전부병례균경수술병리화(혹)림상증실.결과 5례안광NF-1중3례위로광구통종류(기중1례반유상검액부종류),2례위상검、섭、액부종류.전부존재불동정도적로광골이상,주요표현:접골대익결실5례,광상렬증대5례,광벽골질결실1례,접안변형1례,섭골변형1례.령외,4례환자상응부위주망막낭종.결론 안광NF-1대부분존재불동정도적로광골이상,이접골대익결실최위현저.기발생궤제복잡,가능동시존재선천발육이상화계발성개변,유대림상연구.
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.