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Corneal stromal granular dystrophy type 1 Groenouw: case presentation

Poster Details

First Author: M.Radenkovic SERBIA

Co Author(s):    G. Stankovic-Babic   P. Jovanovic   M. Trenkic Bozinovic   M. Zivkovic   B. Dzunic   A. Knezevic

Abstract Details

Purpose:

Corneal dystrophies are a group of rare, non-inflammatory, bilateral hereditary disorders characterized by pathognomonic patterns of corneal deposition and morphological changes. There are over 20 dystrophies affecting all parts of the cornea, classified by International Committee for Classification of Corneal Dystrophies (IC3D) into: epithelial-subepithelial, epithelial-stromal dystrophies, stromal and endothelial dystrophies. Stromal corneal dystrophies are classified based on their phenotypic appearance at the slit lamp: granular, lattice, and macular. Corneal opacities caused by genetic mutations in TGFβ1 gene on chromosome 5q31, leads to transcription and accumulation of aberrant, insoluble protein-keratoepithelin. Primarily affects stroma, over time extends anteriorly or to Descemet’s membrane.

Setting:

These type of granular stromal corneal dystrophy is diagnosed clinically, during slit lamp ophthalmologic examination.

Methods:

ZV(55,female) during routine ophthalmological checking of visual acuity and intraocular pressure 3 years ago, discrete central cornal cloudings were noticed. Main complain photophobia. VOD=cc -3,0Dsph=0,9-1,0; VOS=cc -4,0Dsph= 0,8-0,9 TOU= 11mmHg; Slit lamp examination: bilateral multiple, small, gray-white oval opacities in visual axis separated by clear areas, not-extended into peripheral cornea. Localisation by narrow slit lamp beam beneath Bowman layer and anterior stroma is apparent. Nuclear sclerosis of lens. Refractometry: ROD= -6.0/-4,0(162) ROS= -9,0/ -1,50(10) Keratometry: OD= 42,62/45,37 OS=44,12/45,87 Tear BUT: OD=11s OS=8s Shirmer test: OU>15mm FOU: Conus myopicus

Results:

According to presentation, rare stromal granular corneal dystrophy type I was presumed in myopic eyes, with atypical late presentation in fifth decade of life. After years of regular control slight corneal changes occur, showing slowly progression and late onset, without affecting visual acuity. Artefitial drops were recommended to prolong tear film breakup time and prevent recurrent corneal erosions. Although corneal biopsy was not performed histological structure of deposits was not confitmed, but it is supposed hyaline material to be stained by Masson trichrome .

Conclusions:

Granular corneal dystrophy is uncommon worldwide, type I is more common in Europe, with good prognosis and different onset. Late onset and slow progression is presented in these case. In early phase only medical treatment is recommended: artefitial tears and hypertonic saline solution 5% to prevent erosions. Sometimes anterior segment optical coherence tomography (OCT) could be used to guide phototherapeutic keratectomy (PTK) to remove superfitial opacities. Prospectively if visual acuity worsens because of deep opacities, lamellar or full-thickness corneal transplantation can be performed. Although the success rate is high, granular deposits could recur in graft with time. FINANCIAL DISCLOUSRE: NONE

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