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We read the article by Alur et al.[1] with a great interest. We congratulate the authors and thank them for sharing their experiences with us. Congenital keratoglobus refer to as an association with connective tissue disorders such as Ehlers Danlos syndrome type VI, Marfan syndrome, and Rubinstein-Taybi syndrome.[2] Ehler Danlos syndrome is an autosomal dominant connective tissue disorder caused by defective collagen synthesis. It is categorized by genetic mutations and clinical features. Hyperelasticity and vulnerability of the skin and joints, and friability of the eyes and vessels are the characteristic features.[3] Keratokonus and keratoglobus, which are postulated as ‘Oculus Fragilis’, are the main features of Ehler Danlos type VI. The diagnosis is confirmed based on the abnormal synthesis of the fibroblasts type III procollagen molecules or based on the identification of genetic mutation for type III procollagen (COL3A1). Ehlers-Danlos type VI has a close relationship with a deficiency of lysyl hydroxylase in cultured fibroblasts.[4]

A giant pulmonary artery aneurysm associated with congenital keratoglobus