An Ophthalmological Study of Ectopia Lentis, With Multifarious Aetiopathogenesis, Diverse Clinical Presentations and Varied Prognoses

Abstract

BACKGROUND Ectopia lentis is a hereditary or acquired displacement or malposition of the lens, from its normal position, with traumatic and non-traumatic aetiopathogenesis like homocystinuria due to cystathionine ß-synthase deficiency-chromosome 21q22 mutation, Marfan's syndrome; presenting with varying degrees of lenticular subluxation and manifesting near normal post-operative prognoses. MATERIALS AND METHODS It was a prospective study done on both the eyes of 4 patients (Cases A, B, C, D), which presented to Rajarajeswari Medical College and Hospital, Bangalore, with the complaints of diminution of vision. Case A also complained of progressive headache. Case B and case C gave history of past trauma. They were evaluated with detailed history of presentation and other significant past history. Detailed first visit examination was done, which included vision testing, anterior segment evaluation with slit lamp biomicroscopy and dilated fundus evaluation. They were admitted for further management. Relevant investigations and radiological investigations were done. Orthopaedic, ENT, Dental and Physician opinions were taken for systemic manifestations. Refractive corrections were done by manual small incision cataract surgery with anterior chamber intraocular lens or scleral fixated intraocular lens implantation or lensectomy with anterior vitrectomy and postoperative outcomes were recorded. RESULTS Case A showed 6/36 and 6/24 right and left eye vision, bilateral superonasal subluxation of lens with oval, sluggishly reactive pupil, early cataractous changes, iridodonesis and phacodonesis; with Marfanoid features and increased homocysteine levels. Case B showed visual acuity of 6/24 and counting fingers from 2 m in right and left eye respectively. Right eye was pseudophakic and left inferonasal subluxated lens, hypermature cataract, zonular dehiscence and phacodonesis. Case C showed right 6/60 and left 6/6 vision, with right inferonasally dislocated cataractous lens and phacodonesis. Case D showed right 6/60 and left 6/24 vision, bilateral inferiorly dislocated cataractous lens. After the surgical procedures, the patients recovered to almost near normal vision and ophthalmological conditions. CONCLUSION Case A was diagnosed as ectopia lentis due to homocystinuria, case B and case C as traumatic ectopia lentis and case D as idiopathic ectopia lentis. On proper refractive surgeries and further monitoring, all cases showed excellent post-operative prognoses and recovered well.