先天性小眼症患者佩戴义眼的临床效果

Phil Kyu Lee, W. Cho, ji-Sun Paik, Suk-Woo Yang
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引用次数: 0

摘要

目的:报告先天性小眼症患者接受义眼佩戴等非手术治疗的长期临床效果:方法:对 20 名患者的病历进行回顾性分析。2008年5月至2022年12月期间,共有21只眼睛在韩国首尔圣玛丽医院被诊断为先天性小眼球:本研究共纳入 20 名患者(12 男 8 女),确诊时平均年龄为 4 个月。观察到的眼部畸形包括两例先天性白内障、一例后胚胎瘤、一例角膜虹膜股、四例虹膜巨瘤、五例中央角膜混浊、一例彼得氏畸形和一例球后囊肿。同时还发现了全身性异常,如侧脑室萎缩、胼胝体萎缩、动脉导管未闭、房间隔缺损和语言发育障碍。遗传异常包括抗 SS-A/Ro 抗体阳性、抗核抗体滴度为 1:100,以及通过新一代测序发现的 PAX6 基因突变。未发现特殊家族史或与妊娠相关的因素。平均随访时间为5.94年(范围:1个月至18年),平均角膜直径为4.6毫米,平均轴长为17.44毫米,首次人工眼球试验的平均年龄为5.96岁(范围:7个月至19岁),人工眼球置换的平均间隔时间为22个月(范围:4个月至5年8个月):20名先天性小眼球患者通过定期更换和增大义眼,逐渐扩大了义眼的体积,没有出现严重的并发症。这种方法为患者带来了美学和情感上的积极成果。
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Clinical Results of Prosthetic Eye Wear in Patients with Congenital Microphthalmos
Purpose: To report the long-term clinical outcomes of non-surgical treatment involving prosthetic eye wear in patients diagnosed with congenital microphthalmos.Methods: A retrospective review of the medical records of 20 patients was conducted. In total, 21 eyes were diagnosed with congenital microphthalmos between May 2008 and December 2022 at Seoul St. Mary’s Hospital in Korea.Results: This study included 20 patients (12 males and 8 females) with an average age of 4 months at diagnosis. The observed ophthalmic anomalies included two cases of congenital cataract, one of posterior embryotoxon, one of corneo-iris strand, four of iris coloboma, five of central corneal opacity, one of Peter's anomaly, and one of retrobulbar cyst. Accompanying systemic abnormalities were noted, such as lateral ventricle atrophy, corpus callosum atrophy, patent ductus arteriosus, atrial septal defect, and developmental language disorder. Genetic anomalies included anti SS-A/Ro antibody positivity, a 1:100 titer of anti-nuclear antibody, and a PAX6 mutation identified through next-generation sequencing. No specific family histories or pregnancy-related factors were noted. The average follow-up duration was 5.94 years (range: 1 month to 18 years), the average corneal diameter was 4.6 mm, the average axial length was 17.44 mm, the average age for first artificial eye trial was 5.96 years (range: 7 months to 19 years), and the average interval for artificial eye replacement was 22 months (range: 4 months to 5 years and 8 months).Conclusions: Twenty patients with congenital microphthalmos underwent gradual expansion of their prosthetic eyes by regular replacement and size increase without severe complications. This approach led to aesthetically and emotionally positive outcomes for the patients.
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