François P, Puech B, Turut P
Service d'Exploration Fonctionnelle de la Vision, Lille.
Ophtalmologie. 1990 Jul-Aug;4(4):372-6.
X-shaped macular dystrophy with flavimaculatus flecks is individualized of other heredo-macular dystrophies. This aspect was showed in two families with a retinal pigment epithelial dystrophy characterised by an X-shaped yellowish macular lesion and numerus flavimaculatus retinal flecks. Nine members were variously affected. The condition was bilateral, had a dominant inheritance, started in middle age with a slow-developing macular lesion. Visual functions were often minimally disturbed for two or three decades. Relations with others here-domacular dystrophy are discussed particularly with pattern dystrophy.
伴有黄斑区黄色斑点的X形黄斑营养不良有别于其他遗传性黄斑营养不良。这一特征在两个患有视网膜色素上皮营养不良的家族中得以体现,其特点为黄斑区有X形淡黄色病变以及众多黄斑区视网膜黄色斑点。九名家庭成员受到不同程度的影响。该病为双侧性,呈显性遗传,始于中年,黄斑病变发展缓慢。在长达二三十年的时间里,视觉功能通常仅受到轻微影响。本文特别讨论了它与其他遗传性黄斑营养不良的关系,尤其是与图案性营养不良的关系。