Posters

Polychromatic pre-descemet dystrophy, corneal biomecanics and specular mycroscopy, case reports

Poster Details

First Author: F.Zavarse Fadul SPAIN

Co Author(s):    A. Chapinal López   B. García Valcárcel                 

Abstract Details

Purpose:

To describe possible anatomical and functional alterations with slit lamp examination, specular microscopy and Corneal Biomechanics study in patients with Polychromatic Pre-Descemet Dystrophy (PPDD).

Setting:

Hospital General Universitario Gregorio Marañón, Madrid, Spain.

Methods:

Retrospective, descriptive study, 4 Patients with PPDD with a mean age of 44.75, 3 women and 1 man, blood tests discarding high cholesterol, uric acid and gammopathies, ophthalmological examination including visual acuity, slit-lamp examination, specular microscopy (Topcon SP-P1) and Corneal Biomechanics Study (With Corvis ST).

Results:

Four patients, with a mean age of 44.75, 3 women and 1 man, visual acuity of 0.9(OD) and 0.92(OS). Slit-lamp-biomicroscopy observed punctate, polychromatic, non-confluent, diffusely distributed opacities in the posterior cornea in every patient; also in anterior lens and posterior capsule in one patient. Specular-microscopy revealed a mean CD 3138.7(OD) and 3113.5(OS)cell/mm2, a mean CV 35.75%(OD) and 36.25%(OS); finally, a mean Hexagonality 31.75%(OD) and 28.25%(OS) Corneal Biomechanics: IOP of 15.50(OD) and 15.75(OS), corrected-IOP 13.9(OD) and 14.18(OS). Also the mean CBI index of 0.085(OD) and 0.142(OS), BAD-D 0.905(OD) and 1.305(OS), TBI 0.143(OD) and 0.198(OS), with altered indexes in two patients.

Conclusions:

Polychromaric pre-Descemet dystrophies are still not a well-defined clinical entity, unclear genetic inheritance pattern, asymptomatic patients without vision impairment; presenting Pre-Descemet, thin, focal opacities, without aggregates, leaving greater transparent spaces in-between. Differential diagnosis include stromal deposits (macular dystrophy), guttae (endothelial dystrophy), mucopolysaccharidosis and gammopathies deposits. Even if PPDD does not seem to require treatment over time, it is important to correctly recognize, hence avoiding confusion with other conditions that do require. Recent studies elucidate possible stromal alterations (hyperrreflective keratocytes) and genetic mutations associated with protein function impairment (i.e. PRDX3); therefore finding altered Corneal Biomechanics is a possible outcome.

Financial Disclosure:

None