Ocular manifestations of Hutchinson-Gilford-Progeria syndrome: A rare presentation

Authors : Chandana Sultana, Sharah Rahman, Sarwar Alam, Syeed Mehbub Ul Kadir, Abdul Muntakim Shahid, Rashed Alam, A S M Moinuddin

DOI : 10.18231/j.ijceo.2022.060

Volume : 8

Issue : 2

Year : 2022

Page No : 298-302

The Hutchinson-Gilford Progeria (HGP) syndrome is an exceptionally rare genetic condition characterized by premature and accelerated aging in children. It is demonstrated by developmental delay along with progressive degenerative changes of the integumentary, musculoskeletal, cardiac, and vascular systems. In this case report, we describe the ocular manifestations of Hutchinson-Gilford Progeria (HGP) syndrome of a 20-year-old Bangladeshi boy. The patient had the classic triad of prominent eyes, loss of eyebrows or madarosis and lagophthalmos, which are the most common ocular manifestations.He also developed dry eye, keratinized ocular surface, Meibomian gland dysfunction, vascularized cornea, symblepharon, corneal opacification, and cataract. He had several systemic manifeastations that included senile facies, prominent scalp veins, generalized alopecia with plucked bird appearance, and sclerodermatous changes.
 

Keywords: Hutchinson­gilford, Progeria, Premature, Corneal keratinization, Loss of eyebrow

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