Anwar S, Bradshaw K, Vivian A J
Department of Ophthalmology, Addenbrookes Hospital, Cambridge, UK.
Ophthalmic Genet. 1998 Jun;19(2):81-6. doi: 10.1076/opge.19.2.81.2324.
Trisomy 8 mosaicism can present with a varied clinical picture. A significant number of cases have ocular manifestations. The most commonly reported in the literature have been corneal abnormalities and strabismus. We present a case of trisomy 8 mosaic syndrome with very different ophthalmic manifestations, some of which are previously unreported in the literature. The patient who was known to have trisomy 8 mosaic syndrome was referred with concerns about his visual abilities. He had a characteristic facial dysmorphism and other systemic features associated with this condition. Ophthalmic features included bilateral Duane's syndrome, bilateral myopic astigmatism, congenital pendular nystagmus, and macula hypoplasia. Electrodiagnostic tests confirmed extensive abnormality of cone function in both eyes. This case is discussed in relation to a review of the literature.
8号染色体三体嵌合型可表现出多样的临床症状。相当一部分病例有眼部表现。文献中最常报道的是角膜异常和斜视。我们报告一例8号染色体三体嵌合综合征,其眼部表现非常不同,其中一些在文献中此前未被报道。已知患有8号染色体三体嵌合综合征的患者因视力问题前来就诊。他有典型的面部畸形及与此病症相关的其他全身特征。眼部特征包括双侧杜安综合征、双侧近视散光、先天性摆动性眼球震颤和黄斑发育不全。电诊断测试证实双眼视锥细胞功能广泛异常。结合文献回顾对该病例进行了讨论。