Pomeranz Howard D, Agadzi Anthony K, Ekesten Björn
Department of Ophthalmology, University of Minnesota Medical School, Minneapolis, Minnesota, USA.
Acta Ophthalmol Scand. 2006 Feb;84(1):140-4. doi: 10.1111/j.1600-0420.2005.00523.x.
An 18-month-old white boy, observed by his parents at 1-2 months age to have poor visual attentiveness and nystagmus, underwent an ophthalmological evaluation. The patient also underwent unsedated 5-channel flash visual evoked potentials (VEP) and sedated electroretinogram (ERG) testing as well as magnetic resonance imaging (MRI) of the brain and orbits. The VEP in response to monocular stimulation demonstrated occipital asymmetry and was clearly suggestive of crossed asymmetry and also showed right optic nerve hypoplasia. The MRI and fundoscopic examinations supported the findings of achiasmia and probable optic nerve hypoplasia. The patient also had decreased Teller card visual acuity, nystagmus and a variable right esotropia. Neurological examination was normal. The ophthalmological and MRI findings in this 18-month-old male patient support the diagnosis of isolated non-decussating retinal-fugal fibre syndrome as well as hypoplasia of the optic nerve.
一名18个月大的白人男孩，其父母在他1至2个月大时观察到他视觉注意力差且有眼球震颤，遂接受了眼科评估。该患者还接受了未使用镇静剂的5通道闪光视觉诱发电位（VEP）和使用镇静剂的视网膜电图（ERG）检查，以及脑部和眼眶的磁共振成像（MRI）检查。对单眼刺激的VEP显示枕叶不对称，明显提示交叉不对称，还显示右侧视神经发育不全。MRI和眼底检查支持了无交叉视路和可能的视神经发育不全的发现。该患者还存在泰勒视力卡视力下降、眼球震颤和可变的右眼内斜视。神经系统检查正常。这名18个月大男性患者的眼科和MRI检查结果支持孤立性非交叉视网膜-视路纤维综合征以及视神经发育不全的诊断。
