CaseWise 14

Surgical Management of Ectopia Lentis

Dr. Swati Mishra, Dr. Sarang Lambat

Introduction

Ectopia lentis is defined as an acquired or hereditary lens displaced from its natural position because of defects in the zonular filament.It may occur as an ocular manifestation of a hereditary systemic disorder like Marfan’s syndrome, homocystinuria, Weill-Marchesani syndrome, hyperlisinemia, and sulfite oxidase deficiency. It may also occur without other systemic abnormalities such as idiopathic ectopia lentis. It commonly induces a large refractive error, a partially phakic or aphakic visual axis, and/or anisometropia. In young children, these visual disturbances, if left untreated, can induce dense amblyopia.(1)

Case details

A female patient 10 years of age, came with the complaint of difficulty in reading since past 5 years. Her unaided visual acuity was 6/36 in right eye(RE) and counting finger (CF) 3m in left eye (LE). She was not accepting any correction. Anterior segment examination showed iridodonesis in both eyes (BE). Equator of the lens was visible at the edge of pupil, showing supero-temporal subluxation of clear crystalline lens in BE. (Fig1) Fundus examination was normal. Axial length was 22.5 mm in RE and 23.03 mm in LE.

Management

After detailed systemic and ophthalmic examination a diagnosis of ectopia lentis not associated with any systemic conditions, was made. She had a subluxation of 7 clock hours in BE. We planned a single stage procedure – bilateral Lens aspiration + Capsular segment + Capsular tension ring + PCIOL  in BE for her. She underwent the same first for RE then for LE. (Fig 2) Post-operatively she had improved to 6/12 in RE with a correction of -0.5 DS and +1.75 DC and 6/9 in LE with a correction of +0.50 DC.  Examination of mother showed similar subluxation in BE. She had never been examined earlier. (Fig 3)

Discussion

Ectopia lentis can lead to diplopia, strabismus, and impaired vision which warrants early intervention. Spectacles or contact lenses may be offered for patients but this may be associated with a relatively high possibility of developing amblyopia.(2) Long term complications include zonular weakness and lens instability which is complicated by the loss of the capsular bag, vitreous disturbance, and endothelial cell damage.  Surgical advances have improved patient outcomes in recent years with the inclusion of various techniques including pars plana vitrectomy (PPV) with lensectomy, sulcus fixation of intraocular lenses (IOLs), iris-sutured IOLs, iris-claw IOLs, scleral-sutured capsular tension rings (CTRs) with IOL placement, sutureless sulcus IOL placements, and anterior chamber IOL (AC IOL) placements.(3) Subluxated lens extraction in these patients is challenging and complications include intra-ocular hemorrhage, IOL dislocation, transient rise in IOP, endophthalmitis and retinal detachment.(4) Post operative residual refractive error can be corrected with glasses or contact lenses. In our case, in the bag placement of IOL caused minimal vitreous disturbance which may help in reducing the chances of future retinal complications

Reference

1.Cionni R., Osher R., Marques D. , Marques F. , Snyder M. , Shapiro S.: Modified capsular tension ring for patients with congenital loss of zonular support. J Cataract Refract Surg 2003; 29:1668–1673. DOI:10.1016/S0886-3350(03)00238-4 2.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. DOI:10.4103/meajo. MEAJO_105_16 3.Byrd J., Young M., Liu W., Zhang Y., Tate D.,  Crandall A., Owen L.: Long-term outcomes for pediatric patients having transscleral fixation of the capsular bag with intraocular lens for ectopia lentis. J Cataract Refract Surg . 2018 May ; 44(5): 603–609. DOI:10.1016/j.jcrs.2018.02.016. 4.Esfandiari H.; Ansari S., Mohammad-Rabei H., Mets M. : Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective. J Ophthalmic Vis Res 2019; 14 (1): 71‑77. DOI:  10.4103/jovr.jovr_29_18

× Hello!
%d bloggers like this: