Dagi Linda R, Walton David S
Children's Hospital Boston, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
J AAPOS. 2006 Aug;10(4):345-50. doi: 10.1016/j.jaapos.2006.01.218.
To describe the clinical features of an atypical presentation of ectopia lentis consisting of primary anterior axial lens subluxation in childhood, associated progressive myopia, and complicating angle-closure glaucoma; to facilitate early diagnosis of this triad; and to report the results of lensectomy for treatment.
A retrospective case review of eight patients. Clinical course, visual acuity, gonioscopy, axial length, refraction, fundus examination, associated systemic features, and results of treatment are provided.
This subgroup of patients with ectopia lentis is at risk for progressive, synechial, angle-closure glaucoma. Identification of a clinical triad consisting of rapidly increasing myopia, normal axial length, and progressive axial subluxation leads to earlier diagnosis. Prophylactic lensectomy provides a safe and sight-saving treatment that arrests and, at times, reverses the progression of angle-closure glaucoma.
Ectopia lentis with anterior axial subluxation and progressive myopia can be a sight-threatening condition when significant forward mobilization of the lens results in synechial angle-closure glaucoma. Recognition of this clinical entity can expedite diagnosis and prevent irreversible loss of vision secondary to glaucoma. Because the primary mechanism of angle closure is angle crowding, peripheral iridotomy does little to control the rise in intraocular pressure. Prophylactic lensectomy has proven to be the most reliable treatment in our experience.
描述晶状体异位的一种非典型表现的临床特征，该表现包括儿童期原发性前轴性晶状体半脱位、相关的进行性近视以及并发的闭角型青光眼；促进对这一三联征的早期诊断；并报告晶状体切除术的治疗结果。
对8例患者进行回顾性病例分析。提供了临床病程、视力、前房角镜检查、眼轴长度、验光、眼底检查、相关全身特征以及治疗结果。
这一亚组晶状体异位患者有发生进行性、粘连性闭角型青光眼的风险。识别由快速进展的近视、正常眼轴长度和进行性轴性半脱位组成的临床三联征可实现更早诊断。预防性晶状体切除术提供了一种安全且能挽救视力的治疗方法，可阻止并有时逆转闭角型青光眼的进展。
当晶状体显著向前移位导致粘连性闭角型青光眼时，伴有前轴性半脱位和进行性近视的晶状体异位可能会威胁视力。认识这一临床实体可加快诊断并防止青光眼导致的不可逆视力丧失。由于闭角的主要机制是房角拥挤，周边虹膜切开术对控制眼压升高作用不大。根据我们的经验，预防性晶状体切除术已被证明是最可靠的治疗方法。
