儿童Marfan综合征的临床表现和眼部并发症的防治

目的 分析马凡综合征的临床表现和眼部并发症的防治情况。方法 对儿童16例马凡综合征的临床资料进行回顾性分析。结果 16例患者中晶状体脱位占100％，视网膜脱离占12．5％。心血管异常占43．75％。体形高瘦、蛛状指占50％，有家族史占31％。行晶状体摘除和人工晶状体植入术后。患儿视力均得到不同程度的提高。结论 对可疑马凡综合征的儿童。应及时全身检查，对相关项目综合分析。脱位品状体的早期摘除和人工晶状体植入可有效防止患儿青光眼、色素膜炎和弱视并发症的发生。

Clinical symptom of Marfan syndrome and preventive treatment of ophthalmic complications

Objective Clinical symptom of Marfan syndrome and preventive treatment of ophthalmic complications were researched.Methods Clinical data of 16 cases of Marfan syndrome were regressively analyzed.Results 100% of 16 patients had subluxated or dislocated lenses;12.5% had retinal detachment;43.75% suffered cardiovasuclar abnormality;all the patients looked high and thin and 50% had clubbed finger,31 % cases had family history.Visual acuity was improved in all the cases after lens extraction and intraocular lens implantation.Conclusions Children who suspected with Marfan syndrome should have earlier general examination.And earlier lens extraction and intraocular lens implantation is useful for patients to prevent ophthalmic complications such as glaucoma,uvitis and amblyopia.