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色素性视网膜炎伴发性晶状体异位:病例报告及文献复习。

Spontaneous Ectopia Lentis in Retinitis Pigmentosa: A Case Report and Review of the Literature.

机构信息

Eye Clinic, Neuromuscular and Sense Organs Department, Careggi University Hospital, 50134 Florence, Italy.

Department of Neurosciences, Psychology, Drug Research and Child Health, University of Florence, 50121 Florence, Italy.

出版信息

Medicina (Kaunas). 2024 Aug 8;60(8):1281. doi: 10.3390/medicina60081281.

Abstract

: We report the successful surgical treatment of a case of spontaneous complete anterior crystalline lens luxation in a patient affected by retinitis pigmentosa (RP), associated with elevated intraocular pressure and pupillary block. Additionally, we review the current literature regarding the association between ectopia lentis and RP. : A 44-year-old female RP patient presented to our emergency department reporting severe ocular pain in her left eye (LE) and sickness. She had no history of ocular trauma and did not report systemic disorders. The best corrected visual acuity at presentation was 1/20 in her LE, the intraocular pressure was 60 mmHg, and slit lamp examination showed in her LE a complete dislocation of the lens in the anterior chamber, with mydriasis, atalamia, and a pupillary block. The patient had been administered intravenous mannitol 18% solution and dorzolamide-timolol eye drops and was hospitalized for urgent lens extraction. Anterior segment optical coherence tomography and ultrasound biomicroscopy were performed before surgery. Decompressive 23-gauge pars plana vitrectomy and phacoemulsification were performed, and the capsular bag was removed due to marked zonular weakness, with deferred intraocular lens implant. : Acute angle closure glaucoma in patients with RP may be rarely caused by spontaneous anterior lens dislocation. To our knowledge, this is the first report of spontaneous anterior lens dislocation in an RP patient, documented through photographs, anterior segment optical coherence tomography, and ultrasound biomicroscopy.

摘要

我们报告了一例成功治疗的病例,一名患有色素性视网膜炎(RP)的患者发生自发性完全性前晶状体晶状体脱位,伴有眼内压升高和瞳孔阻滞。此外,我们回顾了目前关于晶状体异位与 RP 之间关联的文献。

一名 44 岁的女性 RP 患者因左眼(LE)剧烈疼痛和不适到我院急诊科就诊。她没有眼部外伤史,也没有报告过系统疾病。就诊时她的最佳矫正视力为左眼 1/20,眼压为 60mmHg,裂隙灯检查显示左眼晶状体完全脱位至前房,散瞳、眼前段和瞳孔阻滞。患者已接受 18%静脉注射甘露醇溶液和多佐胺-噻吗洛尔滴眼液治疗,并住院紧急进行晶状体摘除。手术前进行了眼前段光学相干断层扫描和超声生物显微镜检查。行 23G 经睫状体平坦部减压玻璃体切除术和超声乳化术,由于明显的环状弱化,晶状体囊袋被去除,延期植入人工晶状体。

RP 患者的急性闭角型青光眼可能很少由自发性前晶状体脱位引起。据我们所知,这是首例通过照片、眼前段光学相干断层扫描和超声生物显微镜记录的 RP 患者自发性前晶状体脱位的报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2aa4/11356699/cfdd92f3e2a2/medicina-60-01281-g001.jpg

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