Background: Congenital hereditary endothelial dystrophy is a rare dystrophy in which there's focal or diffuse thickening of Descemet membrane and endothelial degeneration.
Case presentation: A 65-year-old male patient was presented with congenital hereditary endothelial dystrophy. The right eye was operated for penetrating keratoplasty (PKP) twice because of graft failure. On examination, the left eye showed generalized opacification of the cornea, the lens was cataractous and pupil didn't dilate more than 3 mm with topical tropicamide and phenylephrine.
Conclusion: The patient was managed with combined PKP with cataract extraction (open sky) and using iris hooks during surgery to address a small pupil.
Key words: CHED, penetrating keratoplasty, phacoemulsification, iris hook
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