9/6/2023 0 Comments Ectopic pupil definition![]() ![]() Arrest in development or abnormal insertion of secondary lens fibers both of which may be secondary to a nutritional deficiency from defects in tunica vasculosa lentis and may occur in the fifth and sixth month of embryonic life as the lens is normally spherical at this stage.Later on hyaloid degenerations are noted due to changes in shape of lens affecting the lens fibers.įollowing mechanisms has been suggested : ![]() ![]() In addition there may be abnormal development and distribution of secondary lens fibers. In histopathological study it has been found that zonular fibers may be abnormally long and somewhat rudimentary. Pattern of inheritance is mainly autosomal recessive, but it can also be autosomal dominant. Rhizomelic form of Chondrodysplasia punctataįamilial microspherophakia is not associated with systemic abnormalities. Microspherophakia may occur as an isolated defect, may be familial or it might be associated with systemic / local anomalies as follows:Įntities associated with microspherophakia It might have a role in regulation of elastic fibre assembly.ĪDAMTS17 is part of same family of metalloproteinases as ADAMTS10 which is the main gene associated with Weill-Marchesani syndrome. LTBPs are a family of proteins showing structural homologies with fibrillins and is expressed in trabecular meshwork, ciliary processes and lens capsule/epithelium layer. Latent TGF β-binding protein-2 (LTBP2) gene.Mutations in following genes have been noted: May be associated with lens dislocation/ subluxation, high myopia, defective accommodation, glaucoma.Movement of lens with change in posture.Equator of the lens is visible with full mydriasis.Increased anteroposterior diameter of the lens.Microspherophakia has the following characteristics: ![]()
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