Familial Steep Corneas And Refractive Myopia In Posterior Polymorphous Corneal Dystrophy
Published 2022 - 40th Congress of the ESCRS
Reference: FPS06.02 | Type: Free paper | DOI: 10.82333/b5ad-hj23
Authors: Oriel Spierer* 1 , Eli Neimark 1
1Ophthalmology,Wolfson Medical Center, Sackler Faculty of Medicine, Tel Aviv University,Tel Aviv,Israel
Purpose
Posterior polymorphous corneal dystrophy (PPMD) is a rare, autosomal dominant bilateral corneal dystrophy. We present four family members with PPMD, steep corneas and myopia.
Setting
A case series of 4 patients.
Methods
Three siblings with PPMD were referred to our clinic. The patients underwent a comprehensive eye examination and ancillary tests. Their mother was also known to have PPMD.
Results
Examination of an 11-months old female revealed bilateral diffuse corneal opacities, vesicular aggregates at the Descemet membrane and endothelial stippling, compatible with PPMD. Cycloplegic refraction was -18.00/+3.00×180 in the right eye and -17.00/+3.00×180 in the left eye. Keratometry readings were 69.5/66.7 D in the right eye and 67.3/66.1 D in the left eye. Axial lengths were normal to age. Her 4.5 years old sister was also diagnosed with PPMD. Refraction was -12.75/+2.75×90 in the right eye and -12.00/+2.50×85 in the left eye. Keratometry readings were 58.2/55.9 D in the right eye and 58.1/54.8 D in the left eye. The 7 years old brother and their mother had also bilateral PPMD, moderate to high myopia and mildly steep corneas.
Conclusions
PPMD may uncommonly be associated with refractive high myopia due to steep corneas. Descemet membrane collagen abnormality might be the cause of the increased corneal curvature.