Giuffré G
J Fr Ophtalmol. 1985;8(8-9):565-72.
The tilted disc syndrome is an ocular anomaly characterized by dysversion of the optic nerve head, congenital crescent, vessel anomalies, ectasia and depigmentation of the infero-nasal fundus. Its pathogenesis is not known, but there are many theories as to its origin. The most convincing one links the tilted disc syndrome to an anomalous closing of the embryonic fissure. However this hypothesis does not adequately explains the origin of all the anomalies found in the syndrome. In this paper a new theory is suggested. The ganglion cell fibres of the infero-nasal retina could be misdirected toward the orbit through an anomalous embryonic fissure. In this way the fibres could not reach their central connections and degenerate. The hypoplasia of the infero-nasal quadrant of the eye could originate from the anomalous closing of the embryonic fissure and from the atrophy of the ganglion fibres; the crescent from the lack of fibres in the inferior optic disc; the anomalous appearance of the optic nerve head and the retinal vessels from a shifting of the disc upward due to the imbalance between the ganglion cell fibres coming from the superior and inferior retina.
倾斜盘综合征是一种眼部异常,其特征为视神经乳头错位、先天性半月形、血管异常、鼻下眼底扩张和色素脱失。其发病机制尚不清楚,但关于其起源有许多理论。最有说服力的一种理论将倾斜盘综合征与胚胎裂的异常闭合联系起来。然而,这一假说并不能充分解释该综合征中发现的所有异常的起源。本文提出了一种新理论。鼻下视网膜的神经节细胞纤维可能通过异常的胚胎裂向眼眶方向错误引导。这样,纤维就无法到达其中心连接并退化。眼睛鼻下象限的发育不全可能源于胚胎裂的异常闭合和神经节纤维的萎缩;半月形是由于视神经盘下部缺乏纤维;视神经乳头和视网膜血管的异常外观是由于来自上下视网膜的神经节细胞纤维失衡导致盘向上移位。