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Goldenher 综合症在临床上很少见。1978年—1982年我们在临床上遇到四例,现就典型的二例报告如下:临床特征:goldenher 综合症又名眼——耳发育不良(oculo—auriculardysplasia);也有人称作眼—耳—椎骨发育不良(oculo—auricular—vertebraldysplasia)。临床上的主要特征为眼球表面的皮样囊肿或脂肪皮样囊肿,付耳,耳前瘘管及脊椎发育异常。目前病因不清楚,有人认为是先天遗传性疾病,但未得到证实。染色体的研究也没有结论。眼部特征:眼部最常见的为皮样囊肿或脂肪皮样囊肿。有的病人还有上睑缺损,小眼球及其他眼部组织缺损。个别的有小泪点阻塞,眼肌麻卑,以及 Duane 后
Goldenher syndrome is clinically rare. From 1978 to 1982, we encountered four cases clinically. The following two typical reports are as follows: Clinical features: Goldenher syndrome is also called oculo-auricular dysplasia; also called eye-ear - Vertebral dysplasia (oculo-auricular-vertebral esplasia). Clinically the main features of the surface of the dermoid cyst or fatty dermoid cysts, ear, ear fistula and spine dysplasia. At present the cause is not clear, some people think it is a congenital genetic disease, but has not been confirmed. Chromosome studies have not concluded. Eye features: The most common eye for dermoid cyst or fatty dermoid cysts. Some patients also have blepharops, small eyeballs and other ocular tissue defects. Individual small tears clogged, eye muscle numb, as well as Duane after