Towbin R, Garcia-Revillo J, Fitz C
Department of Radiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA.
Pediatr Radiol. 1998 Dec;28(12):995-7. doi: 10.1007/s002470050518.
A 4-year-old boy with bilateral optic sheath enlargement and progressive optic atrophy and blindness is presented. Computed tomography demonstrated hydrocephalus and enlargement of the optic nerve sheath complex. The child died during an attempted repair of hypoplastic atrioventricular valves. Autopsy demonstrated a patulous perioptic subarachnoid space and optic atrophy. This condition has been described in the literature but has not had radiologic-pathologic correlation. With the availability of magnetic resonance imaging, this diagnosis may be made prospectively, thus, it is important for the radiologist to be aware of this entity because optic atrophy and blindness may be prevented by early diagnosis and surgery.
本文报告了一名4岁男孩，其双侧视神经鞘增大，并伴有进行性视神经萎缩和失明。计算机断层扫描显示脑积水以及视神经鞘复合体增大。该患儿在尝试修复发育不全的房室瓣时死亡。尸检显示视神经周围蛛网膜下腔扩张和视神经萎缩。这种情况在文献中已有描述，但尚未有放射学与病理学的相关性研究。随着磁共振成像技术的应用，可以前瞻性地做出这一诊断，因此，放射科医生了解这一疾病实体很重要，因为早期诊断和手术可能预防视神经萎缩和失明。
