Birkebaek Niels Holtum, Patel Leena, Wright Neville Bryce, Grigg John Russell, Sinha Smeeta, Hall Catherine Margaret, Price David Anthony, Lloyd Ian Christopher, Clayton Peter Ellis
Department of Pediatrics, Aarhus University Hospital at Skejby, Denmark.
J Pediatr. 2004 Oct;145(4):536-41. doi: 10.1016/j.jpeds.2004.06.041.
To objectively define criteria for intracranial optic nerve (ON) size in ON hypoplasia (ONH) on magnetic resonance imaging (MRI) scans.
Intracranial ON sizes from MRI were compared between 46 children with ONH diagnosed by ophthalmoscopy (group 1, isolated ONH, 8 children; and group 2, ONH associated with abnormalities of the hypothalamic-pituitary axis and septum pellucidum, 38 children) and children with multiple pituitary hormone deficiency (group 3, multiple pituitary hormone deficiency, 14 children), isolated growth hormone deficiency (group 4, isolated growth hormone deficiency, 15 children), and idiopathic short stature (group 5, idiopathic short stature, 10 children). Intracranial ON size was determined by the cross-sectional area, calculated as [pi x (1/2) height x (1/2) width].
Groups 1 and 2 had lower intracranial ON size than did groups 3, 4, and 5 (P < .001). No patients in groups 3 through 5 who had MRI after 12 months of age (when 95% adult size of ONs is attained) had ONs <2.9 mm 2 . Visual acuity correlated significantly with ON size (P < .01).
Magnetic resonance imaging of the ONs with cross-sectional area <2.9 mm 2 in a short child more than 12 months of age, with or without hypothalamic-pituitary axis abnormalities, confirms the clinical diagnosis of ONH.
客观确定磁共振成像（MRI）扫描中视神经发育不全（ONH）患者颅内视神经（ON）大小的标准。
比较46例经检眼镜诊断为ONH的儿童（第1组，孤立性ONH，8例儿童；第2组，ONH合并下丘脑 - 垂体轴及透明隔异常，38例儿童）与多垂体激素缺乏患儿（第3组，多垂体激素缺乏，14例儿童）、孤立性生长激素缺乏患儿（第4组，孤立性生长激素缺乏，15例儿童）及特发性矮小患儿（第5组，特发性矮小，10例儿童）的MRI颅内ON大小。颅内ON大小通过横截面积确定，计算公式为[π×（1/2）高×（1/2）宽]。
第1组和第2组的颅内ON大小低于第3组、第4组和第5组（P <.001）。12个月龄后（此时ON达到成人大小的95%）进行MRI检查的第3至5组患者中，没有ON<2.9 mm² 的情况。视力与ON大小显著相关（P <.01）。
12个月龄以上儿童，无论有无下丘脑 - 垂体轴异常，若MRI显示ON横截面积<2.9 mm² ，可确诊ONH。
