ISSN: 2578-465X
Authors: Syeed Mehbub Ul Kadir
Aim: To describe the clinical profiles of patients of three successive generations of a Bangladeshi family affected by familial ectopia lentis. Methods: A case series study was conducted in a tertiary eye hospital in Bangladesh from 2015 to 2019. We evaluated twentythree people of the same family of three successive generations. Among them, thirteen patients were affected by familial ectopia lentis. Several patients from all three generations underwent elaborate general examinations to rule out systemic and metabolic disorders. Results: Among thirteen patients, eleven had bilateral Ectopia lentis. Among six adult patients, three had bilateral cataracts. ICCE was done on one patient from 2nd generation (pedigree 7) in another eye hospital. The other two patients (Pedigree-2 and 9) were blind; among seven child patients, four developed cataracts. One was visually significant and underwent PPL with AVT (Pedigree-14). Visual rehabilitation was done with aphakic glass. The other three children had cataracts but not in the pupillary area - not visually significant. No systemic associations were found except one who was suspected of Marfan syndrome. Conclusions: Not only many patients in one family but also the occurrence in three generations is exceptional for Ectopia Lentis Syndrome. The autosomal recessive mode was found in other studies, but this family pedigree analysis favoured an autosomal dominant inheritance. Emphasis is needed on the importance of regular follow-up and management.
Keywords: Ectopia Lentis; Hypermetropia; Subluxation; Aphakic; Pars Plana Lensectomy
