Cross H E
Birth Defects Orig Artic Ser. 1976;12(3):335-46.
Ectopia lentis continues to be a perplexing diagnostic and therapeutic problem for most ophthalmologists. However, thorough investigation, both systemic and ocular, can in most cases, establish either an environmental etiology or the nature of the heritable disorder. In cases with systemic disease and ectopia lentis, the ophthalmologist should insure that each patient obtains adequate consultation with other specialists in order that proper prophylactic and therapeutic measures are available. The Marfan syndrome, homocystinuria, and the Weill-Marchesani together account for the majority of dislocated lenses. Each of these requires a somewhat different therapeutic approach. Glaucoma should be recognized as a frequent complication in each of these conditions but is most serious in the Weill-Marchesani syndrome in which it often occurs early in life and remains unrecognized until serious damage has occurred. Because of the pupillary-block nature of the glaucoma in this condition, a prophylactic peripheral iridectomy must be seriously considered in every case while lens surgery should be undertaken if the glaucoma cannot be controlled by medical or surgical means. In homocystinuria, because of the potential vascular complications of general anesthesia, the ophthalmologist is often in a dilemma. The lens in this condition seems to be more mobile than in other ectopia lentis disorders and therefore likely to compromise vision earlier in life. However, due to the risk of vascular thrombosis, lens surgery should be delayed whenever possible until the patient is old enough to tolerate local anesthesia. Likewise, patients with the Marfan syndrome should be handled conservatively and, in general, function quite well simple with spectacle correction. Due to the high frequency of immediate surgical complications, it is advisable to avoid an open-sky technique to lens removal, using aspiration or a pars plana approach instead.
晶状体异位对于大多数眼科医生来说仍然是一个令人困惑的诊断和治疗难题。然而，通过全面的系统和眼部检查，在大多数情况下可以确定环境病因或遗传性疾病的性质。对于患有全身性疾病和晶状体异位的患者，眼科医生应确保每位患者都能得到其他专科医生的充分会诊，以便采取适当的预防和治疗措施。马凡综合征、同型胱氨酸尿症和韦尔-马歇桑尼综合征共同构成了晶状体脱位的主要原因。每种疾病都需要略有不同的治疗方法。青光眼应被视为这些疾病中常见的并发症，但在韦尔-马歇桑尼综合征中最为严重，该病常发生于生命早期，在造成严重损害之前往往未被察觉。由于这种情况下青光眼的瞳孔阻滞性质，对于每个病例都必须认真考虑预防性周边虹膜切除术，而如果青光眼无法通过药物或手术手段控制，则应进行晶状体手术。在同型胱氨酸尿症中，由于全身麻醉存在潜在的血管并发症，眼科医生常常陷入两难境地。这种情况下的晶状体似乎比其他晶状体异位疾病中的晶状体更易活动，因此可能在生命早期就影响视力。然而，由于存在血管血栓形成的风险，只要有可能，晶状体手术应推迟到患者年龄足够大能够耐受局部麻醉时进行。同样，对于马凡综合征患者应采取保守治疗，一般来说，单纯佩戴眼镜矫正就能很好地发挥功能。由于立即进行手术并发症的发生率很高，建议避免采用开放式晶状体摘除技术，而采用抽吸或睫状体平坦部入路。
