[2] While Macular Corneal Dystrophy is found throughout the world, countries with the highest prevalence include Iceland, Saudi Arabia, India, and the United States.

[6] Macular Corneal Dystrophy is an autosomal recessive genetic disorder caused by mutations in the carbohydrate sulfotransferase gene (CHST6), resulting in abnormal proteoglycan synthesis.

[2] While some individuals remain asymptomatic, initial symptoms typically consist of painful attacks with photophobia, foreign body sensations, and recurrent erosions.

[7] Various imaging modalities, including confocal microscopy and ocular coherence tomography, can provide information about the changes within the cornea and may be suitable replacements for tissue biopsy and excision.

While corneal transplant has traditionally been the standard treatment, less-invasive surgical techniques such as deep anterior lamellar keratoplasty and photo-therapeutic keratectomy are increasingly playing a role in management of MCD.