Abstract
A 19-year-old male with congenital hereditary endothelial dystrophy (CHED) presented with severe bilateral corneal clouding precluding any view of the intraocular structures. He underwent modified Descemet's stripping automated endothelial keratoplasty (DSAEK) technique including a suture pull-through technique to prevent lens damage. Surgery resulted in progressive clearing of the cornea and decreased corneal thickness. Visual acuity increased from hand motions preoperatively to counting fingers at 4 m after 4 months. DSAEK can be successfully performed in phakic eyes with CHED as an alternative to penetrating keratoplasty. It has the advantage of less wound problems and better preservation of globe integrity especially in children.
Highlights
Congenital hereditary endothelial dystrophy (CHED) is a rare corneal dystrophy causing bilateral diffuse corneal clouding due to an abnormal endothelium and Descemet’s membrane (DM).[1]
Major reasons for delaying PKP in children with CHED include low scleral rigidity and positive vitreous pressure resulting in a higher rate of intraoperative complications, suture induced astigmatism and amblyopia, suture complications, need for multiple examinations under anesthesia (EUA), and traumatic wound dehiscence.[2]
CHED is a primary disease of the corneal endothelium
Summary
Modified Descemet’s Stripping Automated Endothelial Keratoplasty for Congenital Hereditary Endothelial Dystrophy.
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