|Year : 2023 | Volume
| Issue : 1 | Page : 195-196
Nontraumatic bilateral inferior ectopia lentis
Piergiacomo Grassi, Yashin Ramkissoon
Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield University Hospitals NHS Foundation Trust, Sheffield, United Kingdom
|Date of Submission||15-Dec-2019|
|Date of Acceptance||12-Mar-2022|
|Date of Web Publication||21-Feb-2023|
Senior Lecturer, School of Medical Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Oxford Road, Manchester, M13 9PL; Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield University Hospitals NHS Foundation Trust, Glossop Road, Sheffield, S10 2JF
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Grassi P, Ramkissoon Y. Nontraumatic bilateral inferior ectopia lentis. Oman J Ophthalmol 2023;16:195-6
An11-year-old myopic boy with left esotropia and amblyopia and no previous ocular and head trauma presented with deteriorating vision and bilateral lens subluxation causing index myopia and astigmatism. His right best-corrected visual acuity (BCVA) had declined from 6/12 to 6/18 (spherical equivalent [SE] −10.75 D), and left BCVA remained 6/36 (SE −9.00 D). Examination showed bilateral marked phacodonesis of crystalline lenses subluxed inferiorly [Figure 1] and [Figure 2]. Bilateral vitreolensectomies were performed with postoperative aphakia and contact lenses (CLs) correction, with a recovery of BCVA to 6/7.5 right (SE +13.00) and final left VA 6/50 (SE +12.50) at 1 year. Systemic workup showed mild learning disabilities only.
|Figure 1: Right eye anterior segment photographs showing marked phacodonesis of right crystalline lens dislocated inferiorly|
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|Figure 2: Left eye anterior segment photographs showing marked phacodonesis of left crystalline lens dislocated inferiorly|
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| Questions|| |
- What is the most likely diagnosis based on history, examination, and systemic findings?
- What systemic and ocular disorders need to be considered in the differential diagnosis?
- What are the management options?
| Answers for Clinical Quiz|| |
| Answers|| |
- Marfan syndrome, Weill–Marchesani syndrome, Ehlers–Danlos syndrome, Sturge–Weber syndrome, aniridia, congenital glaucoma, Axenfeld–Rieger syndrome, retinitis pigmentosa, megalocornea, and pseudoexfoliation syndrome.
- Stable correction of refractive error (RE) with spectacles or CLs, bilateral vitreolensectomies with postoperative aphakia and RE correction with spectacles or CLs, or bilateral vitreolensectomies and intraocular lenses (IOLs) implant with different techniques (scleral-fixated IOL and anterior or retropupillary iris–claw IOL).
| Discussion|| |
Ectopia lentis (EL) is a hereditary or acquired displacement of the crystalline lens due to defective zonular filaments. EL may be secondary to trauma,
inflammation, or hypermature cataract; however, genetic etiologies account for up to 80% of cases.
Homocystinuria caused by cystathionine-β-synthase deficiency is an autosomal recessive disorder, resulting in elevated homocysteinemia. Bilateral EL is the main ocular feature in 90% of patients, with decreased zonular integrity resulting from the enzymatic defect, and lenses are usually displaced inferonasally. Systemic findings include intellectual disability, skeletal abnormalities, and increased risk of thrombosis.
The management of EL remains challenging to maximize BCVAs, prevent amblyopia, and correct RE with spectacles, CLs, or IOLs implants. Pars plana vitrectomy is an established treatment for eyes with rhegmatogenous retinal detachment (including challenging cases complicated by proliferative vitreoretinopathy) and with vitreoretinal interface syndrome and with aqueous misdirection.,,,
but less so for different conditions.,,,
Anterior iris–claw or retropupillary iris–claw IOL implant after vitreolensectomy has been suggested in EL eyes without capsular support;,
decrease in the average endothelial cells density and cystoid macular edema are the main long-term complications described. Alternative techniques include scleral-fixated IOLs (postoperative increase in BCVAs in 83% of treated eyes and high incidence of residual RE in 94% of operated eyes have been reported)
and transscleral fixation of capsular bag with capsular tension ring and IOL into the bag (postoperative increase in BCVAs in 78.5% of treated eyes and incidence of posterior capsule opacification in 52% of operated eyes have been reported).
Homocystinuria should be suspected in young children presenting with bilateral inferior EL and learning disabilities. We recommend early surgical treatment if adequate or stable correction of RE is impaired to minimize the risk of amblyopia.Financial support and sponsorship
Nil.Conflicts of interest
There are no conflicts of interest.
| References|| |
Grassi P, Mudhar HS, Cornish KS. Traumatic suprachoroidal dislocation of crystalline lens. Ophthalmol Retina 2020;4:856-8.
Kaliaperumal S, Kumar KP, Bhuvaneshwari. Varied phenotypic presentations of homocystinuria in two siblings. Indian J Ophthalmol 2014;62:93-4.
] [Full text]
Grassi P, Melville S, Hariprasad AS, Winder S, Ramkissoon Y, Spiteri-Cornish K, et al.
Structural and functional macular changes after retinectomy for retinal detachment complicated by proliferative vitreoretinopathy. Retina 2021;41:2531-9.
Foo E, Grassi P, Spiteri-Cornish K. Early vitrectomy in eyes with non-diabetic vitreous hemorrhage. Ther Adv Ophthalmol 2022;14:25158414221090099. doi:10.1177/25158414221090099.
Romano MR, Cennamo G, Grassi P, Sparnelli F, Allegrini D, Cennamo G. Changes in macular pigment optical density after membrane peeling. PLoS One 2018;13:e0197034.
Low S, Mohamed R, Davidson A, Papadopoulos M, Grassi P, Webster AR, et al
. A new paradigm for delivering personalised care: integrating genetics with surgical interventions in BEST1 mutations. Eye (Lond) 2020;34:577-83.
Grassi P, Chawla A, Rundle P. Vitrectomy for vitreous hemorrhage from vitreous operculum over retinocytoma. Ophthalmol Retina 2019;3:1055.
Grassi P. Photodynamic therapy for vasoproliferative tumour associated with retinitis pigmentosa and Usher syndrome type 2. Clin Exp Optom 2022;105:91-3.
Grassi P, Alexander P. Spontaneous resolution of serous macular detachment and outer retinal defect in optic disc pit maculopathy. Clin Exp Optom 2020;103:384-5.
Grassi P, Wang H, Spiteri-Cornish K, Chawla A, Ramkissoon Y, Subramani S, et al
. The effect of COVID-19 on the vitreoretinal service of a tertiary referral centre: Real-world experience from the Royal Hallamshire Hospital. Eur J Ophthalmol 2022;32:NP335-NP338.doi:10.1177/11206721211049717.
Català-Mora J, Cuadras D, Díaz-Cascajosa J, Castany-Aregall M, Prat-Bartomeu J, García-Arumí J. Anterior iris-claw intraocular lens implantation for the management of nontraumatic ectopia lentis: Long-term outcomes in a paediatric cohort. Acta Ophthalmol 2017;95:170-4.
Faria MY, Ferreira N, Neto E. Retropupillary iris-claw intraocular lens in ectopia lentis in Marfan syndrome. Int Med Case Rep J 2016;9:149-53.
Al Somali AI, Al-Dossari FN, Emara KE, Al Habash A. Outcomes of scleral-fixated intraocular-lens in children with idiopathic ectopia lentis. Middle East Afr J Ophthalmol 2017;24:167-70.
Byrd JM, Young MP, Liu W, Zhang Y, Tate DB, Crandall AS, et al.
Long-term outcomes for pediatric patients having transscleral fixation of the capsular bag with intraocular lens for ectopia lentis. J Cataract Refract Surg 2018;44:603-9.
[Figure 1], [Figure 2]