先天性嗅觉功能障碍的MRI表现

目的 研究先天性嗅觉功能障碍患者的嗅觉系统先天发育异常的MRI表现.方法 选择47例先天性嗅觉功能障碍(39例为Kallmann综合征,8例为单纯嗅觉功能障碍)患者和21名健康志愿者行MR检查.观察MRI上颅内异常表现,测最嗅球体积、嗅沟深度、垂体及垂体柄径线.用χ2检验比较2组患者中嗅球嗅束完全不发育比例有无差异,非参数检验比较2组患者存在的嗅球体积的差别,用方差分析比较各组间垂体和垂体柄径线的差异.结果 所有先大性嗅觉功能障碍患者MRI上嗅球、嗅束和(或)嗅沟均有异常表现,可表现为完全不发育或发育不良,双侧可不对称.Kannlann综合征(31/39)较单纯嗅觉功能障碍患者(2/8)更多地表现为嗅球、嗅束不发育(χ2=6.998,P=0.008),嗅球存在者嗅球体积也较小(Kallmann综合征患者存在的嗅球体积中位数为8 mm3,单纯嗅觉功能障碍患者为22 mm3,Z=-2.902,P=0.004).Kalhnann综合征患者垂体较正常志愿者小[Kallmann综合征患者垂体前后径为(7.22±.93)mm,正常志愿者为(9.94±1.59)mm,F=16.835,P=0.000;Kallmann综合征垂体高度为(3.71±1.74)mm,正常志愿者为(6.00±1.24)mm,F=16.092,P=0.000],垂体柄纤细[Kallmalm综合征患者为(1.19±0.55)mm,正常志愿者为(1.88±0.49)mm,F=13.060,P=0.000].结论 MRI可显示先天性嗅觉功能障碍患者嗅球、嗅束、嗅沟的先天发育异常,对临床诊治提供有价值的信息.

MRI findings of congenital dysosmia

Objective To study the MRI findings of congenital dysosmia. Methods Forty-seven patients with congenital dysosmia (39 with Kallmann syndrome and 8 with isolated dysosmia) and 21 normal volunteers underwent MRI examination. The features of congenital malformation were recorded. The volume of olfactory bulbs, depth of olfactory sulei as well as diameters of pituitary glands and stalks were measured. The rate of dysplasia of olfactory bulbs and tracts in the two patients groups was compared with χ2 test. The difference of volume of olfactory bulbs between the two groups was evaluated with nonparametrie test. And the difference of diameters of pituitary glands and stalks was analyzed with analysis of variance. Results All the patients had abnormal findings in olfactory bulbs, tracts and/or olfactory sulci on MR images. The patterns of congenital malformation may be dysplastic or hypoplastic, symmetric or asymmetric. The proportion of patients with dysplasia of olfactory bulbs and tracts in Kallmann syndrome patients ( 31/39 ) was higher than that in isolated dysosmia ones ( 2/8 ) ( χ2= 6. 998, P = 0. 008 ), and the olfactory bulbs' volume of patients with Kallmann syndrome ( median 8 mm3 ) was smaller than that of patients with isolated dysosmia ( median 22 mm3 ) ( Z = - 2. 902, P = 0. 004 ). The pituitary glands were smaller and the stalks were thinner in patients with Kallmann syndrome than those in volunteers [ the anteroposterior diameter of pituitary glands in Kallmann syndrome (7. 22±1.93) mm, that in normal volunteers (9. 94±1.59) ram, F=16.835, P=0. 000; height of pituitary glands in Kallmann syndrome (3.71±1.74) mm, that in normal volunteers (6. 00±1.24) ram, F = 16. 092, P = 0. 000; the anteroposterior diameter of pituitary stalks in Kallmann syndrome ( 1.19±0. 55 ) mm, that in normal volunteers ( 1.88±0. 49 ) mm, F = 13.060, P =0. 000]. Conclusions In congenital dysosmic patients, dysplasia or hypoplasia of olfactory bulbs, tracts and sulei can be clearly depicted on MR images. MR imaging is valuable for clinical diagnosis and treatment.