先天性眼球畸形的CT、MRI表现

CT、MRI Findings of Congenital Eyeball Malformation

目的提高对先天性眼球畸形的CT、MRI表现认识,并探讨其临床诊断价值。资料与方法回顾性分析24例(31眼)先天性眼球畸形患者的CT、MRI资料,其中行眼眶CT检查13例,眼眶MRI9例,颅脑MRI2例。结果牵牛花综合征16眼,特征性表现为眼球后部视神经乳头局限性缺损致玻璃体腔膨出,合并视网膜脱离2眼,合并眼眶囊肿9眼,合并胼胝体发育不全1眼。永存原始玻璃体增殖症(persistent hyperplastic primary vitreous,PHPV)7眼,表现为小眼球,晶状体后方软组织肿块和永存玻璃体动脉。巩膜葡萄肿1眼。Coats病2眼,主要表现为视网膜下积液,球内密度或信号增高。单纯小眼球3眼。先天性无眼球2眼。结论CT、MRI有助于先天性眼球畸形的诊断及鉴别诊断。

Objective To analyse CT and MRI findings of patients with congenital eyeball malformation and to explore the clinic values. Materials and Methods CT and/or MRI findings in 24 patients（ 31 eyes） were analyzed retrospectively. Axial CT scanning were performed in 9 eases, Axial and Coronal CT in 4 cases simultaneously, and MR was performed in 11 cases. Results Coloboma was depicted in 16 eyes, with typical protrusion of vitreous, resulting from focal defect of posterior globe at optic nerve head insertion. Among them retinal detachment was demonstrated in 2 eyes, orbital cyst in 9 eyes and hypoplastic of corpus callosum in 1 eye respectively. PHPV was visualized in 7 eyes with features including microphthalmus, retrolental tissue and persistent hyaloid artery. Coats＂ disease was revealed in 2 eyes with features of hyperdensity substance posterior to the vitreous on CT or hyperintense on T1WI and T2WI MR. Pure microphthalmus was delineated in 3 eyes. Anophthalmus was observed in 2 eyes. Conclusion CT and MR imaging provide valuable information to differentiate congenital eyeball malformation from other orbital pathologies, particularly from retinoblastoma.