Majoulet Alexandre, Audo Isabelle, Goujard Cécile, De Menthon Mathilde, Chaix Fabrice, Safar Pierre, Labetoulle Marc, Rousseau Antoine
Department of Ophthalmology Assistance Publique - Hôpitaux de Paris, Service d'ophtalmologie, Hôpital Bicêtre, 78, Rue du Général Leclerc, 94275, Le Kremlin Bicêtre, France.
INSERM, CNRS, Institut de la Vision, Sorbonne Université, 75012, Paris, France.
Doc Ophthalmol. 2022 Apr;144(2):147-152. doi: 10.1007/s10633-021-09860-w. Epub 2022 Jan 3.
To report a case of typical delayed-onset hypoxic cortical blindness that occurred few days after resuscitation from drowning in a young male.
Neurological and ophthalmological examination were performed including optical coherence tomography (OCT), Goldmann perimetry, pattern electroretinogram (pERG), pattern and flash visual evoked potentials (pVEP and fVEP) and brain magnetic resonance imaging (MRI).
At presentation, at day 12 post-hypoxic incident, best corrected visual acuity (BCVA) was reduced to hand motion OU with an abolished optokinetic nystagmus, a normal fundus and no relative afferent pupillary defect. Macular and peripapillary OCT were normal. Goldmann perimetry revealed bilateral centrocecal scotoma. pERG was normal while pVEPs were undetectable and fVEPs were abnormal with delayed, decreased and disorganized responses, without interhemispheric asymmetry. Brain MRI disclosed a bilateral cortical-subcortical occipital hypersignal with laminar necrosis and thus confirmed the diagnosis of delayed-onset hypoxic cortical blindness. Visual rehabilitation, including visual stimulation in the scotomatous areas, was associated with a dramatic and rapid visual improvement with a BCVA of 20/32 OU, an ability to read after 2 weeks (day 30 post-hypoxic incident), and a reduction in the size of the scotoma.
Delayed-onset hypoxic cortical blindness is a rare presentation of cortical blindness that develops few days after a cerebral hypoxic stress. While initial presentation can be catastrophic, visual improvement may be spectacular and enhanced with visual rehabilitation.
报告一例年轻男性溺水复苏几天后发生的典型迟发性缺氧性皮质盲病例。
进行了神经学和眼科检查，包括光学相干断层扫描（OCT）、戈德曼视野计检查、图形视网膜电图（pERG）、图形和闪光视觉诱发电位（pVEP和fVEP）以及脑磁共振成像（MRI）。
在缺氧事件发生后第12天就诊时，最佳矫正视力（BCVA）降至双眼手动视力，视动性眼球震颤消失，眼底正常，无相对性传入瞳孔障碍。黄斑和视乳头周围OCT正常。戈德曼视野计检查显示双侧中心暗点。pERG正常，而pVEP无法检测到，fVEP异常，反应延迟、减弱且紊乱，无半球间不对称。脑MRI显示双侧枕叶皮质 - 皮质下高信号伴层状坏死，从而确诊为迟发性缺氧性皮质盲。视觉康复，包括在暗点区域进行视觉刺激，与视力显著快速改善相关，BCVA达到双眼20/32，在2周后（缺氧事件发生后第30天）能够阅读，暗点大小减小。
迟发性缺氧性皮质盲是皮质盲的一种罕见表现，在脑缺氧应激几天后出现。虽然最初表现可能很严重，但视力改善可能非常显著，且通过视觉康复可得到增强。
