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Congenital ectopia lentis and secondary buphthalmos likely occurring as an autosomal recessive trait
Author(s) -
Bjerrum K.,
Kessing S. V.
Publication year - 1991
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.1991.tb04851.x
Subject(s) - ectopia lentis , ophthalmology , marfan syndrome , medicine , gonioscopy , distension , lens (geology) , glaucoma , surgery , biology , paleontology
A family from Turkey with congenital ectopia lentis, likely occurring as an autosomal recessive trait, is presented. No systemic disorders such as homocysteinuria, Marfan's or Weill‐Marchesani's syndromes were found in any of the patients. However, all patients except one were less than 2 years old when first examined so that lens luxation must have happened very early in life. Besides almost total lack of zonular threads the patients presented anomalies of ocular dimensions with increased corneal diameters and axial lengths, and in several cases glaucomatous distension of the optic discs could also be found. A possible explanation for these buphthalmic changes and their relation to lens luxation taking place very early in life is proposed. The importance of early diagnosis and treatment is discussed.