Non-traumatic Ectopia lentis in a paediatric ophthalmology practice, Ibadan, Nigeria

Abstract

Background: Non-traumatic ectopia lentis is a rare ocular disorder usually associated with syndromes like Marfans’s syndrome, Weil- Marchesani and Homocystinuria. Ectopia lentis can lead to profound visual loss from refractive errors, glaucoma as well as retinal  detachment if left unattended.

Aim: The aim of this study was to describe the clinical profile of patients with non-traumatic ectopia lentis  in a paediatric ophthalmology clinic in Ibadan, Nigeria.

Methods: The clinical records of children <16 years diagnosed with non- traumatic ectopia lentis at the Paediatric Ophthalmology Clinic, University College Hospital Ibadan, from May 1, 2015 to Dec 31, 2019 were  retrospectively reviewed. Information on demography, family history, visual acuity (VA), mean refractive error (spherical equivalent), and  management was retrieved.

Results: Clinical records of 25 patients were reviewed. The mean age was 8.9 (±3.41) years with a range of  2-15 years. Sixteen (64%) patients were males. All the patients had bilateral involvement. A positive family history of ectopia lentis was elicited in 6 (24%) patients. Thirteen (52%) patients had a Marfanoid habitus and superior displacement of the lens was observed in 26  (52%) eyes. The presenting visual acuity was <6/60 in 22 (44%) eyes. The spherical equivalent of the refractive errors ranged from  -20.00DS to +13.25DS. Twenty (40%) eyes underwent surgery within the period of the study and the best corrected postoperative visual  acuity improved by 2 or more lines in 12 (60%) of operated eyes.

Conclusion: Severe visual morbidity was common in this cohort of  patients with ectopia lentis in our practice. Treatment provided some improvement in vision  which highlights the need to encourage  early presentation for care. Detailed family history is important as a few of the patients were diagnosed following acceptance of our  invitation to siblings for ophthalmic evaluation.